Local Coverage Article Billing and Coding

Billing and Coding: Erythropoiesis Stimulating Agents

A58982

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Article ID
A58982
Article Title
Billing and Coding: Erythropoiesis Stimulating Agents
Article Type
Billing and Coding
Original Effective Date
07/24/2022
Revision Effective Date
10/01/2022
Revision Ending Date
N/A
Retirement Date
N/A
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CMS National Coverage Policy

Title XVIII of the Social Security Act, §1833(e) prohibits Medicare payment for any claim which lacks the necessary information to process the claim.

CMS Internet-Only Manual, Publication 100-02, Medicare Benefit Policy Manual, Chapter 11, §20.3 Drugs and Biologicals, §100.6 Applicability of Specific ESRD PPS Policies to Acute Kidney Injury (AKI) Dialysis.

CMS Internet-Only Manual, Publication 100-02, Medicare Benefit Policy Manual, Chapter 15, §§50.5.2-50.5.2.2 Coverage for Erythropoietin (EPO) including Epoetin Alfa (Procrit) for preoperative use.

CMS Internet-Only Manual, Publication 100-03, Chapter 1, Part 2, §110.21 Erythropoiesis stimulating Agents (ESAs) in Cancer Related Neoplastic Conditions.

CMS Internet-Only Manual, Publication 100-04, Medicare Claims Processing Manual, Chapter 8, §§60.4.1-60.4.4.2 ESA Claims Monitoring Policy and Facility Billing Requirements, §§60.4.6.1-60.4.6.5 Information Required on CMS-1500 Form and Payment Information.

CMS Internet-Only Manual, Publication 100-04, Medicare Claims Processing Manual, Chapter 17, §§80.8-80.12 Erythropoiesis Stimulating Agents (ESAs) Billing Guidance.

Medicare Learning Network (MLN) Matters (MM5818); Effective Date July 30, 2007

Medicare Learning Network (MLN) Matters (MM5699); Effective Date January 1, 2008

Article Guidance

Article Text

The information in this article contains billing, coding or other guidelines that complement the Local Coverage Determination (LCD) for Erythropoiesis Stimulating Agents L39237.

Part A and B:

General Information for Erythropoiesis Stimulating Agents (ESA) claims:

  • It is not appropriate to bill Medicare for services that are not covered as if they are covered. When billing for non-covered services, use the appropriate modifier.
  • For services requiring a referring/ordering physician, the name and NPI of the referring/ordering physician must be reported on the claim.
  • A claim submitted without a valid ICD-10-CM diagnosis code will be returned to the provider as an incomplete claim under Section 1833(e) of the Social Security Act. The diagnosis code(s) must best describe the patient's condition for which the service was performed.
  • No ESA should be given within the context of uncontrolled hypertension.
  • No ESA should be used to replace red blood cell (RBC) transfusions in patients who need immediate urgent correction of anemia.
  • With any ESA, the patient’s medical record should reflect the clinical reason for dose changes and hematocrit (HCT) levels outside the range of 30.0-36.0% (hemoglobin (Hb) levels 10.0-12.0g/dL). Medicare contractors may review medical records to assure appropriate dose reductions are applied and maintained and hematological target ranges are maintained.
  • There may be instances when a patient has a chronic health condition not specifically addressed in this policy for which an ESA is useful treatment. Such scenarios would be expected to demonstrate considerable transfusion dependence and anemia-related symptoms. In the event of denials, an appeals process will allow the opportunity for the provider to substantiate a reasonable and necessary basis for ESA treatment based on medical literature, specialty organization best practice alignment, and the unique clinical circumstances for the beneficiary. Explanatory documentation within the medical record is crucial.

HCPCS Drug Codes:

Healthcare Common Procedure Coding System (HCPCS) codes J0881, J0885, J0888, and Q5106 are for use in patients with non-end stage renal disease (ESRD) conditions.

HCPCS codes J0882, J0887, Q4081, and Q5105 are intended for use only with patients who have ESRD and are on dialysis.

Modifiers:

-EA, EB, EC

All non-ESRD claims reporting HCPCS code J0881, J0885, J0888, or Q5106 and ESRD claims reporting J0882, J0887, Q4081, and Q5105 must report one and only one of the following modifiers:

  • EA: ESA administered to treat anemia due to anticancer chemotherapy
  • EB: ESA administered to treat anemia due to anticancer radiotherapy
  • EC: ESA administered to treat anemia not due to anticancer radiotherapy or anticancer chemotherapy

ESA claims, either institutional or professional, that do not report one of the above three modifiers will be returned to the provider.

Non-ESRD ESA claims that report the ESA modifier EC and any of the following conditions will be denied as not reasonable and necessary:

  • Any anemia in cancer or cancer treatment patients due to:
    • Folate deficiency
    • B-12 deficiency
    • Iron deficiency
    • Hemolysis
    • Bleeding
    • Bone marrow fibrosis
  • Anemia associated with the treatment of acute and chronic myelogenous leukemias (CML, AML) or erythroid cancers
  • Anemia of cancer not related to cancer treatment
  • Prophylactic use to prevent chemotherapy-induced anemia
  • Prophylactic use to reduce tumor hypoxia
  • Patients with erythropoietin (EPO)-type resistance due to neutralizing antibodies
  • Anemia due to cancer treatments if patients have uncontrolled hypertension

Non-ESRD ESA services for J0881, J0885 or Q5106 billed with modifier -EC will be denied in the presence of various secondary ICD-10 codes that are non-covered per CMS.

https://www.cms.gov/Medicare/Coverage/DeterminationProcess/downloads/CR12027.zip

Non-ESRD ESA services for J0881, J0885 and Q5106 are not considered reasonable and necessary within the context of other medical conditions for which resolution would be expected prior to starting or to continue ESA administration. Such conditions would include, but not be limited to: iron/vitamin B12/folate deficiencies, G6PD deficiency, pyridoxine deficiency, various forms of hemolysis, hereditary spherocytosis, and pure red cell aplasias. The presence of any of these conditions would reduce the therapeutic impact and effectiveness of the ESA. Additionally, the presence of an unspecified anemia code suggests appropriate evaluation, to determine the nature of the treated anemia, has not been completed.

Non-ESRD ESA claims that report HCPCS J0881, J0885, J0888 or Q5106 billed with ESA modifier EB (ESA, anemia, radio-induced) will be denied.

Non-ESRD ESA claims that report HCPCS J0881, J0885, J0888 or Q5106 billed with modifier EA (ESA, anemia, chemo-induced) for anemia secondary to myelosuppressive anticancer chemotherapy in solid tumors, multiple myeloma, lymphoma, and lymphocytic leukemia when a Hb 10.0g/dL or greater or HCT 30.0% or greater will be denied.

-JA, JB, JE

Route of administration is important information especially with drugs that can be given multiple different ways. All non-ESRD claims reporting HCPCS code J0881, J0885, J0888, or Q5106 and all ESRD claims reporting J0882, Q4081, and Q5105 must also be reported with one and only one of the following modifiers:

  • JA-Administered intravenously
  • JB-Administered subcutaneously
  • JE-Administered via dialysate

-GA, GX, GY or GZ

An Advance Beneficiary Notice of Noncoverage (ABN) may be used for services which are likely to be non-covered, whether for medical necessity or for other reasons. Non-covered services should be billed with modifier –GA (Waiver of liability statement issued as required by payer policy, individual case) when the provider wants to indicate that it is anticipated Medicare will deny a specific service as not reasonable and necessary and an ABN signed by the beneficiary is on file, with -GX (Notice of liability issued, voluntary under payer policy) when the beneficiary has signed an ABN, and a denial is anticipated based on provisions other than medical necessity, such as statutory exclusions of coverage or technical issues, with -GY (Item or service statutorily excluded, does not meet the definition of any Medicare benefit or, for non-Medicare insurers, is not a contract benefit, or with –GZ (Item or service expected to be denied as not reasonable and necessary) when the provider wants to indicate that it is expected that Medicare will deny a service as not reasonable and necessary and that an ABN has not been signed by the beneficiary, as appropriate.

Claims Reporting-Hemoglobin/Hematocrit

Claims billing for the administration of an ESA (HCPCS codes J0881, J0882, J0885, J0887, J0888, Q4081, Q5105 and Q5106) must report the most recent HCT or Hb reading. Claims not reporting this information will be returned to the provider.

For institutional claims the Hb reading is reported with a value code 48 and a HCT reading is reported with the value code 49.

For professional paper claims, test results are reported in item 19 of the Form CMS-1500 claim form. For electronic claims (837P), providers report the Hb or Hct readings in Loop 2400 MEA segment. The specifics are mEA01=TR (for test results), MEA02=R1 (for Hb) orR2 (for HCT), and MEA03= the test results.

Documentation Requirements

The medical record documentation for patients receiving ESA therapy must support the reasonable and necessary basis for such therapy.

  • All documentation must be maintained in the patient's medical record and made available to the contractor upon request.
  • Every page of the record must be legible and include appropriate patient identification information (e.g., complete name, dates of service[s]). The documentation must include the legible signature of the physician or non-physician practitioner responsible for and providing the care to the patient.
  • The submitted medical record must support the use of the selected ICD-10-CM code(s). The submitted CPT/HCPCS code must describe the service performed.
  • For any ESA, the medical record must reflect that ESA therapy for the individualized patient is reasonable and necessary. The medical record must document the most recent blood pressure and demonstrate reasonable control not in significant excess of a baseline range for a given patient, weight in kilograms, date and results of HCT or Hb level prior to the administration of ESA therapy, evidence of assessment ruling out other causative factors of anemia or, if causative factors are present, that they have been managed and that it is still necessary to initiate ESA The dosage and route of administration must be documented.

ESRD on Dialysis ESA Claims with Modifier EC

For patients with ESRD who are on dialysis, HCPCS codes J0882, J0887, Q4081, and Q5105 are not paid by Medicare Part B. ESAs for ESRD on dialysis are included in the composite rate for the ESRD facility. If an ESA is administered to a patient with ESRD on dialysis by a provider other than the patient’s ESRD provider, the administering provider must submit a claim to the ESRD provider for the service. The administering provider must not submit a claim to Medicare Part B.

Required Documentation Elements:

  • The ESA utilized:
    • Darbepoetin alfa (J0882) or epoetin alfa (Q4081) or epoetin alfa-epbx biosimilar (Q5105) or epoetin beta (J0887)
  • Use of the ESA for specific diagnoses indicating symptomatic anemia of chronic kidney disease (CKD) on dialysis 
  • For patients with ESRD who are on dialysis, a diagnosis of D63.1 and a diagnosis of N18.6 must be billed with HCPCS code J0882, J0887, Q4081, or Q5105. The EC modifier is also required. A JA, JB or JE modifier is required. Inclusion of any other ICD-10 diagnoses on the claim which are non-covered with an EC modifier per NCD 110.21, will preclude payment for the ESA.
  • Use of an appropriate dose, route (IV administration is recommended for use in ESRD), and frequency.
  • DOSING:
    • For initial dosing of darbepoetin alfa in ESRD: 0.45 mcg/kg weekly or 0.75 mcg/kg every two weeks.
    • For initial dosing of epoetin alfa/epoetin alfa-epbx biosimilar in ESRD: 50-100 u/kg TIW or if appropriate for the patient, a weekly dose of 75-150 u/kg SQ/IV Q week
    • A typical therapeutic dose range would be 50-300 u/kg TIW.
    • Maintenance dosing range: 12.5-525 u/kg TIW
    • For initial dosing of epoetin beta in ESRD: 6 mcg/kg body weight administered as a single intravenous or subcutaneous injection once every two weeks.
    • Maintenance dosing: Once the Hb has been stabilized, epoetin beta may be administered once monthly using a dose that is twice that of the every two-week dose and subsequently titrated as necessary.
  • A pre-initiation Hb level < 10 g/dL with ongoing individualized dosing to achieve and maintain Hb levels between 10-12 g/dL.
  • Documentation of dose reduction should be evident as the Hb level approaches 12 (11 in the case of epoetin beta or darbepoetin alfa) or when the Hb level rises by more than 1 g/dL within a two-week period. During therapy, hematological parameters should be monitored at least weekly until the Hb is stable and sufficient to minimize the need for transfusion. Thereafter, monitor Hb at least monthly.
  • For patients whose Hb does not attain a level within the range of 10 to 12 g/dL despite appropriate dose titrations over a 12-week period, documentation should not demonstrate further increases in dose amounts but rather continuation of the lowest dose that will avoid the need for recurrent RBC transfusions. When recurrent RBC transfusions become necessary within the setting of ESA use exceeding 12 weeks, the ESA therapy should be stopped due to lack of responsiveness.
  • Increases in dose should not be made more frequently than once a month. 
  • The maximum number of administrations of epoetin alfa/biosimilar for a billing cycle is 13 times in 30 days and 14 times in 31 days. The maximum number of administrations of darbepoetin for a billing cycle is 5 times in 30/31days.
  • Evaluation and treatment of other causes of anemia (and documentation thereof) must occur pre-ESA initiation and at any time thereafter as needed for lack of responsiveness.
  • Prior to and during ESA therapy, the patient’s iron stores, including transferrin saturation (serum iron divided by iron binding capacity) and serum ferritin, must be evaluated. Transferrin saturation should be at least 20%, and ferritin should be at least 100 ng/mL. Iron stores must also be checked prior to dosage increases. Due to the frequency of concomitant iron deficiency in these patients, even without dosage adjustments iron storage must be checked at least every 3 months during ongoing ESA therapy.
  • If the initial dose of an ESA was administered in another setting (i.e. hospital, in a state outside our jurisdiction, or in another facility); subsequent office-administered ESA claims may prompt a Medicare Administrative Contractor request for documentation regarding the clinical criteria supporting the initial administration as well as the need to continue the ESA. This may require review of outside medical records and confirmation that needed pre-treatment lab results and evaluation were completed appropriately.

For CKD NOT on Dialysis with Modifier EC ESA Claims:

The term “without dialysis” refers to patients that are not on a regular course of maintenance dialysis. For patients who need occasional “rescue dialysis”, it would be appropriate to bill HCPCS code J0881, J0885, J0888, or Q5106 since these patients are not on a regular course of maintenance dialysis.

The National Kidney Foundation's Kidney Disease Outcomes Quality Initiative (NKF-K/DOQI) guidelines rely on NHANES III data analysis regarding a relationship of HCT levels with stage of CKD. Based on this analysis, patients with glomerular filtration rate (GFR) < 30 mL/min/1.73 m2 commonly demonstrated severe anemia with HCT levels < 33%. Thus, a GFR < 45 mL/min/1.73m2 is a reasonable threshold to support ESA administration in conjunction with sufficiently low and symptomatic Hb/HCT levels.

Required Documentation Elements:

  • Serum creatinine and/or GFR
  • The ESA utilized:
    • Darbepoetin alfa (J0881) or epoetin alfa (J0885) or epoetin alfa-epbx biosimilar (Q5106) or epoetin beta (J0888)
  • Use of the ESA for specific diagnoses indicating symptomatic anemia of CKD not on dialysis
  • For patients with CKD who are not on dialysis, a diagnosis code of D63.1 and a diagnosis of I12.0, I13.11, I13.2, N18.32, N18.4, or N18.5 must be billed with HCPCS code J0881, J0885, J0888, or Q5106. The EC modifier is also required. A JA or JB modifier is required. Inclusion of any other ICD-10 diagnoses on the claim which are non-covered with an EC modifier per NCD 110.21, will preclude payment for the ESA.
    • Note: Patients with stage I and II and IIIa CKD do not meet the GFR requirements in coverage criteria for the following combination ICD-10 codes:
      • I12.9
      • I13.0
  • Use of an appropriate dose, route, and frequency.
  • DOSING:
    • For initial dosing of darbepoetin alfa in non dialysis CKD: 0.45 mcg/kg intravenously or subcutaneously every 4 weeks
    • For initial dosing of epoetin alfa/epoetin alfa-epbx biosimilar in non dialysis CKD: 50-100 u/kg TIW or, if appropriate for the patient, a weekly dose of 75-150 u/kg SQ/IV Q week.
    • Maintenance dosing range: A therapeutic dose range would be 50-300 u/kg TIW and more typically, 75 to 150 units/kg/week.
    • For initial dosing of epoetin beta in CKD NOT on dialysis CKD in adult CKD patients who not currently treated with an ESA: 0.6 mcg/kg body weight administered as a single intravenous or subcutaneous injection once every two weeks.
    • Maintenance dosing range: Once the Hb has been stabilized, epoetin beta may be administered once monthly using a dose that is twice that of the every-two-week dose and subsequently titrated as necessary.
  • A pre-initiation Hb level < 10 g/dL with ongoing individualized dosing to achieve and maintain Hb levels between 10-12 g/dL.
  • Documentation of dose reduction should be evident as the Hb level approaches 12 (11 in the case of epoetin beta or 10 with darbepoetin alfa) or when the Hb level rises by more than 1 g/dL within a two-week period. During therapy, hematological parameters should be monitored at least weekly until the Hb is stable and sufficient to minimize the need for transfusion. Thereafter, monitor Hb at least monthly.
  • For patients whose Hb does not attain a level within the range of 10 to 12 g/dL despite appropriate dose titrations over a 12-week period, documentation should not demonstrate further increases in dose amounts but rather continuation of the lowest dose that will avoid the need for recurrent RBC transfusions. When recurrent RBC transfusions become necessary within the setting of ESA use exceeding 12 weeks, the ESA therapy should be stopped due to lack of responsiveness.
  • Increases in dose should not be made more frequently than once a month.
  • Evaluation and treatment of other causes of anemia (and documentation thereof) must occur pre-ESA initiation and at any time thereafter as needed for lack of responsiveness.
  • Prior to ESA therapy, the patient’s iron stores, including transferrin saturation (serum iron divided by iron binding capacity) and serum ferritin, must be evaluated. Transferrin saturation should be at least 20%, and ferritin should be at least 100 ng/mL. Iron stores must also be checked prior to dosage increases. Due to the frequency of concomitant iron deficiency in these patients, even without dosage adjustments, iron storage must be checked at least every 3 months during ongoing ESA therapy.
  • If the initial dose of an ESA was administered in another setting (i.e. hospital, in a state outside our jurisdiction, or in another facility); subsequent office-administered ESAs must still meet all the above-mentioned requirements. This may require review of outside medical records and confirmation that needed pre-treatment lab results and evaluation were completed appropriately.

Anemia due to antineoplastic chemotherapy

This policy does not replace or supersede existing Medicare applicable National Coverage Determination (NCD) 110.21.

Epoetin beta is NOT indicated or covered for treatment of anemia due to cancer chemotherapy.

Required Documentation Elements:

  • The ESA utilized:
    • Darbepoetin alfa (J0881) or epoetin alfa (J0885) or epoetin alfa-epbx biosimilar (Q5106)
  • Use of the ESA for symptomatic anemia due to antineoplastic chemotherapy
  • A diagnosis of a non-myeloid malignancy (solid tumor, multiple myeloma, lymphoma, or lymphocytic leukemia)
  • For HCPCS codes J0881, J0885 or Q5106: ICD-10-CM code D64.81 or D61.810 AND one of the malignancy codes listed in ICD-10 Group 3 MUST be billed together. The EA modifier is also required. A JA or JB modifier is required. Inclusion of any other ICD-10 diagnoses on the claim which are non-covered with an EC modifier per NCD 110.21, except for ICD-10 codes strictly indicating a relationship to adverse effect of antineoplastic drugs or chemotherapy, will preclude payment for the ESA.
  • DOSING:
    • For darbepoetin alfa therapy in anemia due to antineoplastic chemotherapy: 2.25 mcg/kg every week subcutaneously or 500 mcg every 3 weeks until completion of the chemotherapy course.
    • If Hb increases more than 1 g/dL in any 2-week period or if a Hb level is reached that will avoid RBC transfusion, then the dose of darbepoetin alfa should be decreased by 40%.
    • During treatment, if Hb increases by less than 1 g/dL and remains below 10 g/dL after 4 weeks of therapy, the dose of darbepoetin alfa may be increased once by 25%.
    • If there is no response as measured by Hb levels or if RBC transfusions are still required after 8 weeks of therapy, darbepoetin therapy should be stopped. Darbepoetin therapy must cease following completion of a chemotherapy course.
    • For epoetin alfa/epoetin alfa-epbx biosimilar in anemia due to antineoplastic chemotherapy:
    • Initial dosing: 150 Units/kg SC TIW or 40,000 units SC weekly
    • Reduce dose by 25% when Hb reaches a level needed to avoid transfusion or increases > 1 g/dL in any 2-week period.
    • Increase dose by 25%, if there is no reduction in transfusion requirements or the Hb level does not rise > 1 g/dL after 4 weeks of therapy.
    • If after 8 weeks of therapy there is no response as measured by Hb levels or if transfusions are still required, epoetin alfa should be discontinued. Epoetin alfa therapy must cease following completion of a chemotherapy course.
  • ESA treatment duration for each course of chemotherapy includes the 8 weeks following the final dose of myelosuppressive chemotherapy in a chemotherapy regimen.
  • Any ESA administered for anemia in association with cancer chemotherapy will only be covered if the Hb level immediately prior to initiation or maintenance of the ESA treatment is less than 10 g/dL (or the HCT < 30%).
  • An ESA should only be started if there is a minimum of two additional months of planned chemotherapy.
  • ESAs should not be used in a patient with cancer receiving myelosuppressive chemotherapy when the anticipated outcome for the cancer is cure.
  • Hb levels should be monitored on a weekly basis in patients receiving ESA therapy until Hb becomes stable. ESA doses should be titrated as low as possible for each patient to achieve and maintain the lowest Hb level sufficient to avoid the need for blood transfusion.
  • Although no specific serum EPO level can be stipulated above which patients would be unlikely to respond to ESA therapy, treatment of patients with grossly elevated serum EPO levels (e.g., > 200 mUnits/mL) is not recommended.
  • Evaluation and treatment of other causes of anemia must occur pre-ESA initiation and at any time thereafter as needed for lack of responsiveness.
  • Prior to ESA therapy, the patient’s iron stores, including transferrin saturation (serum iron divided by iron binding capacity) and serum ferritin, must be evaluated. Transferrin saturation should be at least 20%, and ferritin should be at least 100 ng/mL. Iron stores must also be checked prior to dosage increases. Even without dosage adjustments, iron storage must be checked at least every 3 months during ongoing ESA therapy.
  • If the initial dose of an ESA was administered in another setting (i.e. hospital, in a state outside our jurisdiction, or in another facility); subsequent office-administered ESAs must still meet all the above-mentioned requirements. This may require review of outside medical records and confirmation that need pre-treatment lab results and evaluation were completed appropriately.

Anemia in Zidovudine Treated HIV infection

Darbepoetin alfa and epoetin beta are NOT indicated for this condition.

For epoetin alfa/epoetin alfa-epbx biosimilar in anemia related to Zidovudine treated HIV:

Epoetin alfa is not indicated for the treatment of anemia in HIV-infected patients due to other factors such as iron or folate deficiencies, hemolysis, or gastrointestinal bleeding, which should be managed appropriately.

Epoetin alfa therapy for anemia related to Zidovudine therapy for HIV must not target a Hb > 12 g/dL.

Required Documentation Elements:

  • Use of the ESA for symptomatic anemia due to AZT therapy
  • Zidovudine therapy at a dose < 4200 mg/week
  • An endogenous baseline pre-transfusion serum EPO level < 500 mUnits/mL
  • A dose titrated to the individual need for to achieve and maintain the lowest Hb level needed to avoid RBC transfusions.
  • The ESA Utilized: 
    • Epoetin alfa (J0885) or epoetin alfa-epbx biosimilar (Q5106)
  • For patients with symptomatic anemia due to adverse impact of AZT, a diagnosis of D61.1 and either B20 or B97.35 must be billed with HCPCS codes J0885 or Q5106. A JA or JB modifier is required. The EC modifier is also required. Inclusion of any other ICD-10 diagnoses on the claim which are non-covered with an EC modifier per NCD 110.21, will preclude payment for the ESA.
  • DOSING:
    • Initial dosing: 100 Units/kg TIW for 8 weeks.
    • Maintenance dosing: After 8 weeks of initial therapy, if the response has not been satisfactory in terms of decreasing RBC transfusion or the Hb level, then the dose may be increased by 50-100 units/kg TIW.
    • Response should be evaluated and documented every 4-8 weeks thereafter with the dose adjusted accordingly by 50 to 100 Units/kg increments TIW. If the Hb exceeds 12 g/dL then the ESA should be stopped until the Hb drops below 11 g/dL at which time it can be restarted at a dose reduced by at least 25%. During maintenance, dosage variations will depend on the presence of intercurrent infections and variances in Zidovudine dosing.
    • Dosing should never exceed 300 units/kg TIW as further improvements in response are unlikely.

For Anemia in Myelodysplastic Syndromes (MDS)

MDS comprises a range of hematologic malignancies characterized by isolated or multiple cytopenias accompanied by abnormal cellular maturation. Much of the morbidity and death from MDS is due to the cytopenia impacts rather than transformation to AML. There are well supported scenarios in which ESAs can be used as part of the therapeutic regimen in a very low, low or intermediate risk MDS population. These scenarios, for which coverage can be considered, presuming the reasonable and necessary use of ESAs is well documented, are noted below.

Epoetin beta is NOT indicated for treatment of anemia in MDS.

ESA use for symptomatic anemia in MDS can be considered for the following categories:

  • Patients with MDS with very low, low risk (score < 3) per the Revised International Prognostic Scoring System (IPSS-R), or
  • Patients with intermediate risk (IPSS-R score < 3.5), or
  • Patients with MDS with low or intermediate- 1 risk (score of 0-1) per the International Prognostic Scoring System (IPSS), or
  • Patients with very low, low or intermediate risk (score of 0-2) per the WHO Prognostic Scoring System (WPSS)

AND

  • Without del(5q) and serum EPO < 500 mU/mL, or
  • With del(5q) and no chromosome 7 associated abnormalities, on or before starting Lenalidomide with serum EPO < 500 mU/mL

Required Documentation Elements:

  • Symptomatic anemia or transfusion dependence and a Hb < 10 g/dL within one week of the initial ESA treatment
  • The ESA utilized:
    • Epoetin alfa (J0885) or epoetin alfa-epbx biosimilar (Q5106) or darbepoetin (J0881)
  • Weight in kilograms
  • MDS diagnostic bone marrow biopsy with cytogenetic analysis
  • Documented evaluation for other anemia contributing factors such as blood loss, hemolysis, renal failure, medications, nutritional deficiencies, thyroid dysfunction, autoimmune disorders and anemia of chronic disease
  • Evaluation and treatment of other causes of anemia must occur pre-ESA initiation and at any time thereafter as needed for lack of responsiveness.
  • Iron store results pre-ESA use: serum iron/TIBC (TSAT) and ferritin and at least every 3 months during treatment
  • Transferrin saturation should be at least 20%, and ferritin should be at least 100 ng/mL to start or continue ESA without correction of iron deficiency.
  • Serum EPO result < 500 mU/mL
  • Documented IPSS-R result of 3 or less for very low, low risk MDS or documented IPSS-R result of 3.5 or less for an intermediate risk MDS or IPSS score of 0-1 or WPSS score of 0-2.
  • Narrative regarding ongoing response to therapy
  • If the initial dose of an ESA was administered in another setting (i.e. hospital, in a state outside our jurisdiction, or in another facility); subsequent office-administered ESAs must still meet all the above-mentioned requirements. This may require review of outside medical records and confirmation that need pre-treatment lab results and evaluation were completed appropriately.
  • For patients with symptomatic anemia due to low risk categories of MDS (Very low, Low or intermediate risk IPPS-R score < 3.5), one of the following diagnosis codes should be present on the claim; D46.0, D46.1, D46.20, D46.21, D46.22, D46.A, D46.B, D46.C or D46.Z. In the rare case of essential thrombocythemia progressing to refractory anemia due to secondary myelofibrosis, the dual diagnoses of D47.3 and D75.81 should be reported. Waldenstrom macroglobulinemia cases complicated by neoplastic bone marrow infiltration resulting in symptomatic anemia should simply be reported with C88.0. Inclusion of any other ICD-10 diagnoses on the claim which are non-covered with an EC modifier per NCD 110.21, will preclude payment for the ESA. A JA or JB modifier is required.

ESA use for 6-12 weeks would be expected as would RBC transfusion use for symptom management while awaiting an erythroid response.

The ESA regimen should be adjusted to maintain the lowest level of Hb/HCT that is sufficient to avoid RBC transfusions. If the rise of Hb is adequate to avoid transfusions or increases > 1 g/dL in any given two-week period, the dose of epoetin alfa should be reduced by 25% or the dose of darbepoetin alfa can be reduced by 40%. Less frequent dosing would also be an acceptable alternative.

If the response is not adequate after 4 weeks, dose escalation, an increase in dose frequency or addition of a myeloid growth factor would be acceptable.

ESAs should not be continued for more than twelve weeks if no response is observed.

Before beginning ESA therapy risk of thrombosis should be considered. Hypertension must be controlled.

Much higher doses of ESAs are utilized as compared to all the uses noted otherwise in this article. Dosing for epoetin alfa/epoetin alfa-epbx often is in a range of 40,000-60,000 units SQ 1-2 times per week and darbepoetin alfa often in a range of 150-300 mcg SQ every other week.

There is currently no consensus regarding an optimal dose/schedule for ESA administration. This decision is left to the treating oncologist.

Prophylactic Allogeneic Transfusion Reduction Intent in Presurgical Anemia

Darbepoetin alfa and epoetin beta are NOT indicated for this condition.

For epoetin alfa/epoetin alfa-epbx biosimilar in anemia in a presurgical setting:

  • For HCPCS codes J0885 or Q5106: ICD-10-CM codes D63.8 and Z01.818 MUST be billed along with one of the osteoarthritis codes listed in ICD-10 Group 6. The EC modifier is also required. A JA or JB modifier is required; a JB modifier would be consistent with the FDA label indication. Inclusion of any other ICD-10 diagnoses on the claim which are non-covered with an EC modifier per NCD 110.21, will preclude payment for the ESA.

Required Documentation Elements:

  • A presurgical Hb greater than 10 but less than 13 g/dL.
  • A high risk for perioperative blood loss related to planned elective orthopedic hip or knee surgery with an expectation for more than 2 units of blood loss
  • Inability or unwillingness by the patient to donate autologous blood
  • A workup for anemia that suggests anemia of chronic disease
  • Iron supplementation ongoing during the entire course of the ESA
  • Deep vein thrombosis (DVT) prophylaxis ongoing during the entire course of the ESA
  • Dosing: 300 u/kg/day SQ daily for 10 days prior to surgery, on the day of surgery and daily for 4 days following surgery. The other dosing option would be 600 units/kg SQ once weekly for 3 weeks prior to the surgery (21, 14 and 7 days before surgery) plus a 4th dose on the day of surgery.

Coding Information

CPT/HCPCS Codes

Group 1

(4 Codes)
Group 1 Paragraph

For anemia in patients with ESRD (dialysis requiring)

Group 1 Codes
CodeDescription
J0882 INJECTION, DARBEPOETIN ALFA, 1 MICROGRAM (FOR ESRD ON DIALYSIS)
J0887 INJECTION, EPOETIN BETA, 1 MICROGRAM, (FOR ESRD ON DIALYSIS)
Q4081 INJECTION, EPOETIN ALFA, 100 UNITS (FOR ESRD ON DIALYSIS)
Q5105 INJECTION, EPOETIN ALFA-EPBX, BIOSIMILAR, (RETACRIT) (FOR ESRD ON DIALYSIS), 100 UNITS

Group 2

(4 Codes)
Group 2 Paragraph

For anemia in patients with CKD (non-dialysis requiring)

Group 2 Codes
CodeDescription
J0881 INJECTION, DARBEPOETIN ALFA, 1 MICROGRAM (NON-ESRD USE)
J0885 INJECTION, EPOETIN ALFA, (FOR NON-ESRD USE), 1000 UNITS
J0888 INJECTION, EPOETIN BETA, 1 MICROGRAM, (FOR NON ESRD USE)
Q5106 INJECTION, EPOETIN ALFA-EPBX, BIOSIMILAR, (RETACRIT) (FOR NON-ESRD USE), 1000 UNITS

Group 3

(3 Codes)
Group 3 Paragraph

For anemia in patients with antineoplastic chemotherapy induced anemia

Group 3 Codes
CodeDescription
J0881 INJECTION, DARBEPOETIN ALFA, 1 MICROGRAM (NON-ESRD USE)
J0885 INJECTION, EPOETIN ALFA, (FOR NON-ESRD USE), 1000 UNITS
Q5106 INJECTION, EPOETIN ALFA-EPBX, BIOSIMILAR, (RETACRIT) (FOR NON-ESRD USE), 1000 UNITS

Group 4

(2 Codes)
Group 4 Paragraph

For anemia in patients on Zidovudine for HIV disease

Group 4 Codes
CodeDescription
J0885 INJECTION, EPOETIN ALFA, (FOR NON-ESRD USE), 1000 UNITS
Q5106 INJECTION, EPOETIN ALFA-EPBX, BIOSIMILAR, (RETACRIT) (FOR NON-ESRD USE), 1000 UNITS

Group 5

(3 Codes)
Group 5 Paragraph

For symptomatic anemia associated with MDS

Group 5 Codes
CodeDescription
J0881 INJECTION, DARBEPOETIN ALFA, 1 MICROGRAM (NON-ESRD USE)
J0885 INJECTION, EPOETIN ALFA, (FOR NON-ESRD USE), 1000 UNITS
Q5106 INJECTION, EPOETIN ALFA-EPBX, BIOSIMILAR, (RETACRIT) (FOR NON-ESRD USE), 1000 UNITS

Group 6

(2 Codes)
Group 6 Paragraph

For prophylactic allogeneic transfusion reduction intent in presurgical anemia

Group 6 Codes
CodeDescription
J0885 INJECTION, EPOETIN ALFA, (FOR NON-ESRD USE), 1000 UNITS
Q5106 INJECTION, EPOETIN ALFA-EPBX, BIOSIMILAR, (RETACRIT) (FOR NON-ESRD USE), 1000 UNITS

Group 7

(1 Code)
Group 7 Paragraph

Non-Covered CPT/HCPCS Codes

Group 7 Codes
CodeDescription
J0890 INJECTION, PEGINESATIDE, 0.1 MG (FOR ESRD ON DIALYSIS)

CPT/HCPCS Modifiers

Group 1

(10 Codes)
Group 1 Paragraph

N/A

Group 1 Codes
CodeDescription
EA ERYTHROPOETIC STIMULATING AGENT (ESA) ADMINISTERED TO TREAT ANEMIA DUE TO ANTI-CANCER CHEMOTHERAPY
EB ERYTHROPOETIC STIMULATING AGENT (ESA) ADMINISTERED TO TREAT ANEMIA DUE TO ANTI-CANCER RADIOTHERAPY
EC ERYTHROPOETIC STIMULATING AGENT (ESA) ADMINISTERED TO TREAT ANEMIA NOT DUE TO ANTI-CANCER RADIOTHERAPY OR ANTI-CANCER CHEMOTHERAPY
GA WAIVER OF LIABILITY STATEMENT ISSUED AS REQUIRED BY PAYER POLICY, INDIVIDUAL CASE
GX NOTICE OF LIABILITY ISSUED, VOLUNTARY UNDER PAYER POLICY
GY ITEM OR SERVICE STATUTORILY EXCLUDED, DOES NOT MEET THE DEFINITION OF ANY MEDICARE BENEFIT OR, FOR NON-MEDICARE INSURERS, IS NOT A CONTRACT BENEFIT
GZ ITEM OR SERVICE EXPECTED TO BE DENIED AS NOT REASONABLE AND NECESSARY
JA ADMINISTERED INTRAVENOUSLY
JB ADMINISTERED SUBCUTANEOUSLY
JE ADMINISTERED VIA DIALYSATE

ICD-10-CM Codes that Support Medical Necessity

Group 1

(2 Codes)
Group 1 Paragraph

For PATIENTS WITH ESRD ON DIALYSIS-Both ICD-10 diagnoses must be on the claim in order to designate the type of anemia and the stage of kidney disease.

It is the provider’s responsibility to select ICD-10 codes carried out to the highest level of specificity.

The diagnosis codes listed below require the use of the EC modifier (ESA administered to treat anemia not due to anti-cancer radiotherapy or anti-cancer chemotherapy) when submitting claims for HCPCS code J0882, J0887, Q4081, or Q5105. A JA or JB modifier is required.

The following dual ICD-10-CM codes support medical necessity and provide coverage for HCPCS codes: J0882, J0887, Q4081 or Q5105.

D63.1 Anemia in CKD in conjunction with: N18.6 End stage renal disease

Group 1 Codes
CodeDescription
D63.1 Anemia in chronic kidney disease
N18.6 End stage renal disease

Group 2

(7 Codes)
Group 2 Paragraph

For PATIENTS WITH CKD NOT ON DIALYSIS-Dual diagnoses are expected; an ICD-10 code noting anemia of CKD and an ICD-10 code noting the stage of the CKD.

It is the provider’s responsibility to select ICD-10 codes carried out to the highest level of specificity.

The diagnosis codes listed below require the use of the EC modifier (ESA administered to treat anemia not due to anti-cancer radiotherapy or anti-cancer chemotherapy) when submitting claims for HCPCS code J0881, J0885, J0888, or Q5106. A JA or JB modifier is required.

The following dual ICD-10-CM codes support medical necessity and provide coverage for HCPCS code: J0881, J0885, J0888, or Q5106.

D63.1 Anemia in CKD in conjunction with one of the following: 

Group 2 Codes
CodeDescription
D63.1 Anemia in chronic kidney disease
I12.0 Hypertensive chronic kidney disease with stage 5 chronic kidney disease or end stage renal disease
I13.11 Hypertensive heart and chronic kidney disease without heart failure, with stage 5 chronic kidney disease, or end stage renal disease
I13.2 Hypertensive heart and chronic kidney disease with heart failure and with stage 5 chronic kidney disease, or end stage renal disease
N18.32 Chronic kidney disease, stage 3b
N18.4 Chronic kidney disease, stage 4 (severe)
N18.5 Chronic kidney disease, stage 5

Group 3

(1,021 Codes)
Group 3 Paragraph

For PATIENTS WITH SYMPTOMATIC ANEMIA IN THE SETTING OF CHEMOTHERAPY FOR A NON-MYELOID MALIGNANCY

Dual diagnoses are expected:

  • an ICD-10 code noting antineoplastic chemotherapy induced anemia (D64.81) or antineoplastic chemotherapy induced pancytopenia (D61.810) AND
  • an ICD-10 code noting the non-myeloid malignancy.

Note: C92.00-C92.91, C92.Z0-C92.Z2, C93.00-C93.91, C94.00-C94.02, C94.20-C94.82, C95.00-C95.91, or D45 are myeloid malignancies and are excluded from coverage.

It is the provider’s responsibility to select ICD-10 codes carried out to the highest level of specificity.

The diagnosis codes listed below require the use of the EA modifier (ESA administered to treat anemia due to anti-cancer chemotherapy) when submitting claims for HCPCS code J0881, J0885, or Q5106. A JA or JB modifier is required.

The following dual ICD-10-CM codes support medical necessity and provide coverage for HCPCS code: J0881, J0885 or Q5106.

D64.81 Antineoplastic chemotherapy induced anemia or D61.810 Antineoplastic chemotherapy induced pancytopenia in conjunction with one of the following for the non-myeloid malignancy:

Group 3 Codes
CodeDescription
C00.0 - C00.8 Malignant neoplasm of external upper lip - Malignant neoplasm of overlapping sites of lip
C01 Malignant neoplasm of base of tongue
C02.0 - C02.8 Malignant neoplasm of dorsal surface of tongue - Malignant neoplasm of overlapping sites of tongue
C03.0 - C03.1 Malignant neoplasm of upper gum - Malignant neoplasm of lower gum
C04.0 - C04.8 Malignant neoplasm of anterior floor of mouth - Malignant neoplasm of overlapping sites of floor of mouth
C05.0 - C05.8 Malignant neoplasm of hard palate - Malignant neoplasm of overlapping sites of palate
C06.0 - C06.2 Malignant neoplasm of cheek mucosa - Malignant neoplasm of retromolar area
C06.89 Malignant neoplasm of overlapping sites of other parts of mouth
C07 Malignant neoplasm of parotid gland
C08.0 - C08.1 Malignant neoplasm of submandibular gland - Malignant neoplasm of sublingual gland
C09.0 - C09.8 Malignant neoplasm of tonsillar fossa - Malignant neoplasm of overlapping sites of tonsil
C10.0 - C10.8 Malignant neoplasm of vallecula - Malignant neoplasm of overlapping sites of oropharynx
C11.0 - C11.8 Malignant neoplasm of superior wall of nasopharynx - Malignant neoplasm of overlapping sites of nasopharynx
C12 Malignant neoplasm of pyriform sinus
C13.0 - C13.8 Malignant neoplasm of postcricoid region - Malignant neoplasm of overlapping sites of hypopharynx
C14.0 - C14.8 Malignant neoplasm of pharynx, unspecified - Malignant neoplasm of overlapping sites of lip, oral cavity and pharynx
C15.3 - C15.8 Malignant neoplasm of upper third of esophagus - Malignant neoplasm of overlapping sites of esophagus
C16.0 - C16.8 Malignant neoplasm of cardia - Malignant neoplasm of overlapping sites of stomach
C17.0 - C17.8 Malignant neoplasm of duodenum - Malignant neoplasm of overlapping sites of small intestine
C18.0 - C18.8 Malignant neoplasm of cecum - Malignant neoplasm of overlapping sites of colon
C19 Malignant neoplasm of rectosigmoid junction
C20 Malignant neoplasm of rectum
C21.1 - C21.8 Malignant neoplasm of anal canal - Malignant neoplasm of overlapping sites of rectum, anus and anal canal
C22.0 - C22.9 Liver cell carcinoma - Malignant neoplasm of liver, not specified as primary or secondary
C23 Malignant neoplasm of gallbladder
C24.0 - C24.8 Malignant neoplasm of extrahepatic bile duct - Malignant neoplasm of overlapping sites of biliary tract
C25.0 - C25.8 Malignant neoplasm of head of pancreas - Malignant neoplasm of overlapping sites of pancreas
C26.1 Malignant neoplasm of spleen
C30.0 - C30.1 Malignant neoplasm of nasal cavity - Malignant neoplasm of middle ear
C31.0 - C31.8 Malignant neoplasm of maxillary sinus - Malignant neoplasm of overlapping sites of accessory sinuses
C32.0 - C32.8 Malignant neoplasm of glottis - Malignant neoplasm of overlapping sites of larynx
C33 Malignant neoplasm of trachea
C34.01 - C34.02 Malignant neoplasm of right main bronchus - Malignant neoplasm of left main bronchus
C34.11 - C34.12 Malignant neoplasm of upper lobe, right bronchus or lung - Malignant neoplasm of upper lobe, left bronchus or lung
C34.2 Malignant neoplasm of middle lobe, bronchus or lung
C34.31 - C34.32 Malignant neoplasm of lower lobe, right bronchus or lung - Malignant neoplasm of lower lobe, left bronchus or lung
C34.81 - C34.82 Malignant neoplasm of overlapping sites of right bronchus and lung - Malignant neoplasm of overlapping sites of left bronchus and lung
C37 Malignant neoplasm of thymus
C38.0 - C38.2 Malignant neoplasm of heart - Malignant neoplasm of posterior mediastinum
C38.4 - C38.8 Malignant neoplasm of pleura - Malignant neoplasm of overlapping sites of heart, mediastinum and pleura
C40.01 - C40.02 Malignant neoplasm of scapula and long bones of right upper limb - Malignant neoplasm of scapula and long bones of left upper limb
C40.11 - C40.12 Malignant neoplasm of short bones of right upper limb - Malignant neoplasm of short bones of left upper limb
C40.21 - C40.22 Malignant neoplasm of long bones of right lower limb - Malignant neoplasm of long bones of left lower limb
C40.31 - C40.32 Malignant neoplasm of short bones of right lower limb - Malignant neoplasm of short bones of left lower limb
C40.81 - C40.82 Malignant neoplasm of overlapping sites of bone and articular cartilage of right limb - Malignant neoplasm of overlapping sites of bone and articular cartilage of left limb
C41.0 - C41.4 Malignant neoplasm of bones of skull and face - Malignant neoplasm of pelvic bones, sacrum and coccyx
C43.0 Malignant melanoma of lip
C43.111 - C43.122 Malignant melanoma of right upper eyelid, including canthus - Malignant melanoma of left lower eyelid, including canthus
C43.21 - C43.22 Malignant melanoma of right ear and external auricular canal - Malignant melanoma of left ear and external auricular canal
C43.31 - C43.39 Malignant melanoma of nose - Malignant melanoma of other parts of face
C43.4 Malignant melanoma of scalp and neck
C43.51 - C43.59 Malignant melanoma of anal skin - Malignant melanoma of other part of trunk
C43.61 - C43.62 Malignant melanoma of right upper limb, including shoulder - Malignant melanoma of left upper limb, including shoulder
C43.71 - C43.72 Malignant melanoma of right lower limb, including hip - Malignant melanoma of left lower limb, including hip
C43.8 Malignant melanoma of overlapping sites of skin
C4A.0 Merkel cell carcinoma of lip
C4A.111 - C4A.112 Merkel cell carcinoma of right upper eyelid, including canthus - Merkel cell carcinoma of right lower eyelid, including canthus
C4A.121 - C4A.122 Merkel cell carcinoma of left upper eyelid, including canthus - Merkel cell carcinoma of left lower eyelid, including canthus
C4A.21 - C4A.22 Merkel cell carcinoma of right ear and external auricular canal - Merkel cell carcinoma of left ear and external auricular canal
C4A.31 - C4A.39 Merkel cell carcinoma of nose - Merkel cell carcinoma of other parts of face
C4A.4 Merkel cell carcinoma of scalp and neck
C4A.51 - C4A.59 Merkel cell carcinoma of anal skin - Merkel cell carcinoma of other part of trunk
C4A.61 - C4A.62 Merkel cell carcinoma of right upper limb, including shoulder - Merkel cell carcinoma of left upper limb, including shoulder
C4A.71 - C4A.72 Merkel cell carcinoma of right lower limb, including hip - Merkel cell carcinoma of left lower limb, including hip
C4A.8 Merkel cell carcinoma of overlapping sites
C44.01 - C44.09 Basal cell carcinoma of skin of lip - Other specified malignant neoplasm of skin of lip
C44.1121 - C44.1122 Basal cell carcinoma of skin of right upper eyelid, including canthus - Basal cell carcinoma of skin of right lower eyelid, including canthus
C44.1191 - C44.1192 Basal cell carcinoma of skin of left upper eyelid, including canthus - Basal cell carcinoma of skin of left lower eyelid, including canthus
C44.1221 - C44.1222 Squamous cell carcinoma of skin of right upper eyelid, including canthus - Squamous cell carcinoma of skin of right lower eyelid, including canthus
C44.1291 - C44.1292 Squamous cell carcinoma of skin of left upper eyelid, including canthus - Squamous cell carcinoma of skin of left lower eyelid, including canthus
C44.1321 - C44.1322 Sebaceous cell carcinoma of skin of right upper eyelid, including canthus - Sebaceous cell carcinoma of skin of right lower eyelid, including canthus
C44.1391 - C44.1392 Sebaceous cell carcinoma of skin of left upper eyelid, including canthus - Sebaceous cell carcinoma of skin of left lower eyelid, including canthus
C44.1921 - C44.1922 Other specified malignant neoplasm of skin of right upper eyelid, including canthus - Other specified malignant neoplasm of skin of right lower eyelid, including canthus
C44.1991 - C44.1992 Other specified malignant neoplasm of skin of left upper eyelid, including canthus - Other specified malignant neoplasm of skin of left lower eyelid, including canthus
C44.212 - C44.219 Basal cell carcinoma of skin of right ear and external auricular canal - Basal cell carcinoma of skin of left ear and external auricular canal
C44.222 - C44.229 Squamous cell carcinoma of skin of right ear and external auricular canal - Squamous cell carcinoma of skin of left ear and external auricular canal
C44.292 - C44.299 Other specified malignant neoplasm of skin of right ear and external auricular canal - Other specified malignant neoplasm of skin of left ear and external auricular canal
C44.311 - C44.319 Basal cell carcinoma of skin of nose - Basal cell carcinoma of skin of other parts of face
C44.321 - C44.329 Squamous cell carcinoma of skin of nose - Squamous cell carcinoma of skin of other parts of face
C44.391 - C44.399 Other specified malignant neoplasm of skin of nose - Other specified malignant neoplasm of skin of other parts of face
C44.41 - C44.49 Basal cell carcinoma of skin of scalp and neck - Other specified malignant neoplasm of skin of scalp and neck
C44.510 - C44.519 Basal cell carcinoma of anal skin - Basal cell carcinoma of skin of other part of trunk
C44.520 - C44.529 Squamous cell carcinoma of anal skin - Squamous cell carcinoma of skin of other part of trunk
C44.590 - C44.599 Other specified malignant neoplasm of anal skin - Other specified malignant neoplasm of skin of other part of trunk
C44.612 - C44.619 Basal cell carcinoma of skin of right upper limb, including shoulder - Basal cell carcinoma of skin of left upper limb, including shoulder
C44.622 - C44.629 Squamous cell carcinoma of skin of right upper limb, including shoulder - Squamous cell carcinoma of skin of left upper limb, including shoulder
C44.692 - C44.699 Other specified malignant neoplasm of skin of right upper limb, including shoulder - Other specified malignant neoplasm of skin of left upper limb, including shoulder
C44.712 - C44.719 Basal cell carcinoma of skin of right lower limb, including hip - Basal cell carcinoma of skin of left lower limb, including hip
C44.722 - C44.729 Squamous cell carcinoma of skin of right lower limb, including hip - Squamous cell carcinoma of skin of left lower limb, including hip
C44.792 - C44.799 Other specified malignant neoplasm of skin of right lower limb, including hip - Other specified malignant neoplasm of skin of left lower limb, including hip
C44.81 - C44.89 Basal cell carcinoma of overlapping sites of skin - Other specified malignant neoplasm of overlapping sites of skin
C45.0 - C45.7 Mesothelioma of pleura - Mesothelioma of other sites
C46.0 - C46.4 Kaposi's sarcoma of skin - Kaposi's sarcoma of gastrointestinal sites
C46.51 - C46.52 Kaposi's sarcoma of right lung - Kaposi's sarcoma of left lung
C46.7 Kaposi's sarcoma of other sites
C47.0 Malignant neoplasm of peripheral nerves of head, face and neck
C47.11 - C47.12 Malignant neoplasm of peripheral nerves of right upper limb, including shoulder - Malignant neoplasm of peripheral nerves of left upper limb, including shoulder
C47.21 - C47.22 Malignant neoplasm of peripheral nerves of right lower limb, including hip - Malignant neoplasm of peripheral nerves of left lower limb, including hip
C47.3 - C47.8 Malignant neoplasm of peripheral nerves of thorax - Malignant neoplasm of overlapping sites of peripheral nerves and autonomic nervous system
C48.0 - C48.1 Malignant neoplasm of retroperitoneum - Malignant neoplasm of specified parts of peritoneum
C48.8 Malignant neoplasm of overlapping sites of retroperitoneum and peritoneum
C49.0 Malignant neoplasm of connective and soft tissue of head, face and neck
C49.11 - C49.12 Malignant neoplasm of connective and soft tissue of right upper limb, including shoulder - Malignant neoplasm of connective and soft tissue of left upper limb, including shoulder
C49.21 - C49.22 Malignant neoplasm of connective and soft tissue of right lower limb, including hip - Malignant neoplasm of connective and soft tissue of left lower limb, including hip
C49.3 - C49.8 Malignant neoplasm of connective and soft tissue of thorax - Malignant neoplasm of overlapping sites of connective and soft tissue
C49.A1 - C49.A9 Gastrointestinal stromal tumor of esophagus - Gastrointestinal stromal tumor of other sites
C50.011 - C50.012 Malignant neoplasm of nipple and areola, right female breast - Malignant neoplasm of nipple and areola, left female breast
C50.021 - C50.022 Malignant neoplasm of nipple and areola, right male breast - Malignant neoplasm of nipple and areola, left male breast
C50.111 - C50.112 Malignant neoplasm of central portion of right female breast - Malignant neoplasm of central portion of left female breast
C50.121 - C50.122 Malignant neoplasm of central portion of right male breast - Malignant neoplasm of central portion of left male breast
C50.211 - C50.212 Malignant neoplasm of upper-inner quadrant of right female breast - Malignant neoplasm of upper-inner quadrant of left female breast
C50.221 - C50.222 Malignant neoplasm of upper-inner quadrant of right male breast - Malignant neoplasm of upper-inner quadrant of left male breast
C50.311 - C50.312 Malignant neoplasm of lower-inner quadrant of right female breast - Malignant neoplasm of lower-inner quadrant of left female breast
C50.321 - C50.322 Malignant neoplasm of lower-inner quadrant of right male breast - Malignant neoplasm of lower-inner quadrant of left male breast
C50.411 - C50.412 Malignant neoplasm of upper-outer quadrant of right female breast - Malignant neoplasm of upper-outer quadrant of left female breast
C50.421 - C50.422 Malignant neoplasm of upper-outer quadrant of right male breast - Malignant neoplasm of upper-outer quadrant of left male breast
C50.511 - C50.512 Malignant neoplasm of lower-outer quadrant of right female breast - Malignant neoplasm of lower-outer quadrant of left female breast
C50.521 - C50.522 Malignant neoplasm of lower-outer quadrant of right male breast - Malignant neoplasm of lower-outer quadrant of left male breast
C50.611 - C50.612 Malignant neoplasm of axillary tail of right female breast - Malignant neoplasm of axillary tail of left female breast
C50.621 - C50.622 Malignant neoplasm of axillary tail of right male breast - Malignant neoplasm of axillary tail of left male breast
C50.811 - C50.812 Malignant neoplasm of overlapping sites of right female breast - Malignant neoplasm of overlapping sites of left female breast
C50.821 - C50.822 Malignant neoplasm of overlapping sites of right male breast - Malignant neoplasm of overlapping sites of left male breast
C50.911 - C50.912 Malignant neoplasm of unspecified site of right female breast - Malignant neoplasm of unspecified site of left female breast
C50.921 - C50.922 Malignant neoplasm of unspecified site of right male breast - Malignant neoplasm of unspecified site of left male breast
C51.0 - C51.8 Malignant neoplasm of labium majus - Malignant neoplasm of overlapping sites of vulva
C52 Malignant neoplasm of vagina
C53.0 - C53.8 Malignant neoplasm of endocervix - Malignant neoplasm of overlapping sites of cervix uteri
C54.0 - C54.8 Malignant neoplasm of isthmus uteri - Malignant neoplasm of overlapping sites of corpus uteri
C56.1 - C56.2 Malignant neoplasm of right ovary - Malignant neoplasm of left ovary
C57.01 - C57.02 Malignant neoplasm of right fallopian tube - Malignant neoplasm of left fallopian tube
C57.11 - C57.12 Malignant neoplasm of right broad ligament - Malignant neoplasm of left broad ligament
C57.21 - C57.22 Malignant neoplasm of right round ligament - Malignant neoplasm of left round ligament
C57.3 Malignant neoplasm of parametrium
C57.7 - C57.8 Malignant neoplasm of other specified female genital organs - Malignant neoplasm of overlapping sites of female genital organs
C58 Malignant neoplasm of placenta
C60.0 - C60.2 Malignant neoplasm of prepuce - Malignant neoplasm of body of penis
C60.8 Malignant neoplasm of overlapping sites of penis
C61 Malignant neoplasm of prostate
C62.01 - C62.02 Malignant neoplasm of undescended right testis - Malignant neoplasm of undescended left testis
C62.11 - C62.12 Malignant neoplasm of descended right testis - Malignant neoplasm of descended left testis
C63.01 - C63.02 Malignant neoplasm of right epididymis - Malignant neoplasm of left epididymis
C63.11 - C63.12 Malignant neoplasm of right spermatic cord - Malignant neoplasm of left spermatic cord
C63.2 Malignant neoplasm of scrotum
C63.7 - C63.8 Malignant neoplasm of other specified male genital organs - Malignant neoplasm of overlapping sites of male genital organs
C64.1 - C64.2 Malignant neoplasm of right kidney, except renal pelvis - Malignant neoplasm of left kidney, except renal pelvis
C65.1 - C65.2 Malignant neoplasm of right renal pelvis - Malignant neoplasm of left renal pelvis
C66.1 - C66.2 Malignant neoplasm of right ureter - Malignant neoplasm of left ureter
C67.0 - C67.8 Malignant neoplasm of trigone of bladder - Malignant neoplasm of overlapping sites of bladder
C68.0 - C68.8 Malignant neoplasm of urethra - Malignant neoplasm of overlapping sites of urinary organs
C69.01 - C69.02 Malignant neoplasm of right conjunctiva - Malignant neoplasm of left conjunctiva
C69.11 - C69.12 Malignant neoplasm of right cornea - Malignant neoplasm of left cornea
C69.21 - C69.22 Malignant neoplasm of right retina - Malignant neoplasm of left retina
C69.31 - C69.32 Malignant neoplasm of right choroid - Malignant neoplasm of left choroid
C69.41 - C69.42 Malignant neoplasm of right ciliary body - Malignant neoplasm of left ciliary body
C69.51 - C69.52 Malignant neoplasm of right lacrimal gland and duct - Malignant neoplasm of left lacrimal gland and duct
C69.61 - C69.62 Malignant neoplasm of right orbit - Malignant neoplasm of left orbit
C69.81 - C69.82 Malignant neoplasm of overlapping sites of right eye and adnexa - Malignant neoplasm of overlapping sites of left eye and adnexa
C70.0 - C70.1 Malignant neoplasm of cerebral meninges - Malignant neoplasm of spinal meninges
C71.0 - C71.8 Malignant neoplasm of cerebrum, except lobes and ventricles - Malignant neoplasm of overlapping sites of brain
C72.0 - C72.1 Malignant neoplasm of spinal cord - Malignant neoplasm of cauda equina
C72.21 - C72.22 Malignant neoplasm of right olfactory nerve - Malignant neoplasm of left olfactory nerve
C72.31 - C72.32 Malignant neoplasm of right optic nerve - Malignant neoplasm of left optic nerve
C72.41 - C72.42 Malignant neoplasm of right acoustic nerve - Malignant neoplasm of left acoustic nerve
C72.59 Malignant neoplasm of other cranial nerves
C73 Malignant neoplasm of thyroid gland
C74.01 - C74.02 Malignant neoplasm of cortex of right adrenal gland - Malignant neoplasm of cortex of left adrenal gland
C74.11 - C74.12 Malignant neoplasm of medulla of right adrenal gland - Malignant neoplasm of medulla of left adrenal gland
C75.0 - C75.8 Malignant neoplasm of parathyroid gland - Malignant neoplasm with pluriglandular involvement, unspecified
C7A.010 - C7A.012 Malignant carcinoid tumor of the duodenum - Malignant carcinoid tumor of the ileum
C7A.020 - C7A.026 Malignant carcinoid tumor of the appendix - Malignant carcinoid tumor of the rectum
C7A.090 - C7A.098 Malignant carcinoid tumor of the bronchus and lung - Malignant carcinoid tumors of other sites
C7A.1 Malignant poorly differentiated neuroendocrine tumors
C7A.8 Other malignant neuroendocrine tumors
C7B.01 - C7B.09 Secondary carcinoid tumors of distant lymph nodes - Secondary carcinoid tumors of other sites
C7B.1 - C7B.8 Secondary Merkel cell carcinoma - Other secondary neuroendocrine tumors
C76.0 - C76.3 Malignant neoplasm of head, face and neck - Malignant neoplasm of pelvis
C76.41 - C76.42 Malignant neoplasm of right upper limb - Malignant neoplasm of left upper limb
C76.51 - C76.52 Malignant neoplasm of right lower limb - Malignant neoplasm of left lower limb
C76.8 Malignant neoplasm of other specified ill-defined sites
C77.0 - C77.8 Secondary and unspecified malignant neoplasm of lymph nodes of head, face and neck - Secondary and unspecified malignant neoplasm of lymph nodes of multiple regions
C78.01 - C78.02 Secondary malignant neoplasm of right lung - Secondary malignant neoplasm of left lung
C78.1 - C78.2 Secondary malignant neoplasm of mediastinum - Secondary malignant neoplasm of pleura
C78.39 Secondary malignant neoplasm of other respiratory organs
C78.4 - C78.7 Secondary malignant neoplasm of small intestine - Secondary malignant neoplasm of liver and intrahepatic bile duct
C78.89 Secondary malignant neoplasm of other digestive organs
C79.01 - C79.02 Secondary malignant neoplasm of right kidney and renal pelvis - Secondary malignant neoplasm of left kidney and renal pelvis
C79.11 - C79.19 Secondary malignant neoplasm of bladder - Secondary malignant neoplasm of other urinary organs
C79.2 Secondary malignant neoplasm of skin
C79.31 - C79.32 Secondary malignant neoplasm of brain - Secondary malignant neoplasm of cerebral meninges
C79.49 Secondary malignant neoplasm of other parts of nervous system
C79.51 - C79.52 Secondary malignant neoplasm of bone - Secondary malignant neoplasm of bone marrow
C79.61 - C79.62 Secondary malignant neoplasm of right ovary - Secondary malignant neoplasm of left ovary
C79.71 - C79.72 Secondary malignant neoplasm of right adrenal gland - Secondary malignant neoplasm of left adrenal gland
C79.81 - C79.89 Secondary malignant neoplasm of breast - Secondary malignant neoplasm of other specified sites
C80.0 - C80.2 Disseminated malignant neoplasm, unspecified - Malignant neoplasm associated with transplanted organ
C81.01 - C81.09 Nodular lymphocyte predominant Hodgkin lymphoma, lymph nodes of head, face, and neck - Nodular lymphocyte predominant Hodgkin lymphoma, extranodal and solid organ sites
C81.11 - C81.19 Nodular sclerosis Hodgkin lymphoma, lymph nodes of head, face, and neck - Nodular sclerosis Hodgkin lymphoma, extranodal and solid organ sites
C81.21 - C81.29 Mixed cellularity Hodgkin lymphoma, lymph nodes of head, face, and neck - Mixed cellularity Hodgkin lymphoma, extranodal and solid organ sites
C81.31 - C81.39 Lymphocyte depleted Hodgkin lymphoma, lymph nodes of head, face, and neck - Lymphocyte depleted Hodgkin lymphoma, extranodal and solid organ sites
C81.41 - C81.49 Lymphocyte-rich Hodgkin lymphoma, lymph nodes of head, face, and neck - Lymphocyte-rich Hodgkin lymphoma, extranodal and solid organ sites
C81.71 - C81.79 Other Hodgkin lymphoma, lymph nodes of head, face, and neck - Other Hodgkin lymphoma, extranodal and solid organ sites
C81.91 - C81.99 Hodgkin lymphoma, unspecified, lymph nodes of head, face, and neck - Hodgkin lymphoma, unspecified, extranodal and solid organ sites
C82.01 - C82.09 Follicular lymphoma grade I, lymph nodes of head, face, and neck - Follicular lymphoma grade I, extranodal and solid organ sites
C82.11 - C82.19 Follicular lymphoma grade II, lymph nodes of head, face, and neck - Follicular lymphoma grade II, extranodal and solid organ sites
C82.21 - C82.29 Follicular lymphoma grade III, unspecified, lymph nodes of head, face, and neck - Follicular lymphoma grade III, unspecified, extranodal and solid organ sites
C82.31 - C82.39 Follicular lymphoma grade IIIa, lymph nodes of head, face, and neck - Follicular lymphoma grade IIIa, extranodal and solid organ sites
C82.41 - C82.49 Follicular lymphoma grade IIIb, lymph nodes of head, face, and neck - Follicular lymphoma grade IIIb, extranodal and solid organ sites
C82.51 - C82.59 Diffuse follicle center lymphoma, lymph nodes of head, face, and neck - Diffuse follicle center lymphoma, extranodal and solid organ sites
C82.61 - C82.69 Cutaneous follicle center lymphoma, lymph nodes of head, face, and neck - Cutaneous follicle center lymphoma, extranodal and solid organ sites
C82.81 - C82.89 Other types of follicular lymphoma, lymph nodes of head, face, and neck - Other types of follicular lymphoma, extranodal and solid organ sites
C82.91 - C82.99 Follicular lymphoma, unspecified, lymph nodes of head, face, and neck - Follicular lymphoma, unspecified, extranodal and solid organ sites
C83.01 - C83.09 Small cell B-cell lymphoma, lymph nodes of head, face, and neck - Small cell B-cell lymphoma, extranodal and solid organ sites
C83.11 - C83.19 Mantle cell lymphoma, lymph nodes of head, face, and neck - Mantle cell lymphoma, extranodal and solid organ sites
C83.31 - C83.39 Diffuse large B-cell lymphoma, lymph nodes of head, face, and neck - Diffuse large B-cell lymphoma, extranodal and solid organ sites
C83.51 - C83.59 Lymphoblastic (diffuse) lymphoma, lymph nodes of head, face, and neck - Lymphoblastic (diffuse) lymphoma, extranodal and solid organ sites
C83.71 - C83.79 Burkitt lymphoma, lymph nodes of head, face, and neck - Burkitt lymphoma, extranodal and solid organ sites
C83.81 - C83.89 Other non-follicular lymphoma, lymph nodes of head, face, and neck - Other non-follicular lymphoma, extranodal and solid organ sites
C83.91 - C83.99 Non-follicular (diffuse) lymphoma, unspecified, lymph nodes of head, face, and neck - Non-follicular (diffuse) lymphoma, unspecified, extranodal and solid organ sites
C84.01 - C84.09 Mycosis fungoides, lymph nodes of head, face, and neck - Mycosis fungoides, extranodal and solid organ sites
C84.11 - C84.19 Sezary disease, lymph nodes of head, face, and neck - Sezary disease, extranodal and solid organ sites
C84.41 - C84.49 Peripheral T-cell lymphoma, not elsewhere classified, lymph nodes of head, face, and neck - Peripheral T-cell lymphoma, not elsewhere classified, extranodal and solid organ sites
C84.61 - C84.69 Anaplastic large cell lymphoma, ALK-positive, lymph nodes of head, face, and neck - Anaplastic large cell lymphoma, ALK-positive, extranodal and solid organ sites
C84.71 - C84.79 Anaplastic large cell lymphoma, ALK-negative, lymph nodes of head, face, and neck - Anaplastic large cell lymphoma, ALK-negative, extranodal and solid organ sites
C84.A1 - C84.A9 Cutaneous T-cell lymphoma, unspecified lymph nodes of head, face, and neck - Cutaneous T-cell lymphoma, unspecified, extranodal and solid organ sites
C84.Z1 - C84.Z9 Other mature T/NK-cell lymphomas, lymph nodes of head, face, and neck - Other mature T/NK-cell lymphomas, extranodal and solid organ sites
C84.91 - C84.99 Mature T/NK-cell lymphomas, unspecified, lymph nodes of head, face, and neck - Mature T/NK-cell lymphomas, unspecified, extranodal and solid organ sites
C85.11 - C85.19 Unspecified B-cell lymphoma, lymph nodes of head, face, and neck - Unspecified B-cell lymphoma, extranodal and solid organ sites
C85.21 - C85.29 Mediastinal (thymic) large B-cell lymphoma, lymph nodes of head, face, and neck - Mediastinal (thymic) large B-cell lymphoma, extranodal and solid organ sites
C85.81 - C85.89 Other specified types of non-Hodgkin lymphoma, lymph nodes of head, face, and neck - Other specified types of non-Hodgkin lymphoma, extranodal and solid organ sites
C85.91 - C85.99 Non-Hodgkin lymphoma, unspecified, lymph nodes of head, face, and neck - Non-Hodgkin lymphoma, unspecified, extranodal and solid organ sites
C86.0 - C86.6 Extranodal NK/T-cell lymphoma, nasal type - Primary cutaneous CD30-positive T-cell proliferations
C88.2 - C88.8 Heavy chain disease - Other malignant immunoproliferative diseases
C90.00 Multiple myeloma not having achieved remission
C90.02 Multiple myeloma in relapse
C90.10 Plasma cell leukemia not having achieved remission
C90.12 Plasma cell leukemia in relapse
C90.20 Extramedullary plasmacytoma not having achieved remission
C90.22 Extramedullary plasmacytoma in relapse
C90.30 Solitary plasmacytoma not having achieved remission
C90.32 Solitary plasmacytoma in relapse
C91.00 Acute lymphoblastic leukemia not having achieved remission
C91.02 Acute lymphoblastic leukemia, in relapse
C91.10 Chronic lymphocytic leukemia of B-cell type not having achieved remission
C91.12 Chronic lymphocytic leukemia of B-cell type in relapse
C91.30 Prolymphocytic leukemia of B-cell type not having achieved remission
C91.32 Prolymphocytic leukemia of B-cell type, in relapse
C91.40 Hairy cell leukemia not having achieved remission
C91.42 Hairy cell leukemia, in relapse
C91.50 Adult T-cell lymphoma/leukemia (HTLV-1-associated) not having achieved remission
C91.52 Adult T-cell lymphoma/leukemia (HTLV-1-associated), in relapse
C91.60 Prolymphocytic leukemia of T-cell type not having achieved remission
C91.62 Prolymphocytic leukemia of T-cell type, in relapse
C91.A0 Mature B-cell leukemia Burkitt-type not having achieved remission
C91.A2 Mature B-cell leukemia Burkitt-type, in relapse
C91.Z0 Other lymphoid leukemia not having achieved remission
C91.Z2 Other lymphoid leukemia, in relapse
C91.90 Lymphoid leukemia, unspecified not having achieved remission
C91.92 Lymphoid leukemia, unspecified, in relapse
C96.0 - C96.4 Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis - Sarcoma of dendritic cells (accessory cells)
C96.A Histiocytic sarcoma
C96.Z Other specified malignant neoplasms of lymphoid, hematopoietic and related tissue
D00.01 - D00.08 Carcinoma in situ of labial mucosa and vermilion border - Carcinoma in situ of pharynx
D00.1 - D00.2 Carcinoma in situ of esophagus - Carcinoma in situ of stomach
D01.0 - D01.3 Carcinoma in situ of colon - Carcinoma in situ of anus and anal canal
D01.49 Carcinoma in situ of other parts of intestine
D01.5 Carcinoma in situ of liver, gallbladder and bile ducts
D01.7 Carcinoma in situ of other specified digestive organs
D02.0 - D02.1 Carcinoma in situ of larynx - Carcinoma in situ of trachea
D02.21 - D02.22 Carcinoma in situ of right bronchus and lung - Carcinoma in situ of left bronchus and lung
D02.3 Carcinoma in situ of other parts of respiratory system
D03.0 Melanoma in situ of lip
D03.111 - D03.112 Melanoma in situ of right upper eyelid, including canthus - Melanoma in situ of right lower eyelid, including canthus
D03.121 - D03.122 Melanoma in situ of left upper eyelid, including canthus - Melanoma in situ of left lower eyelid, including canthus
D03.21 - D03.22 Melanoma in situ of right ear and external auricular canal - Melanoma in situ of left ear and external auricular canal
D03.39 Melanoma in situ of other parts of face
D03.4 Melanoma in situ of scalp and neck
D03.51 - D03.59 Melanoma in situ of anal skin - Melanoma in situ of other part of trunk
D03.61 - D03.62 Melanoma in situ of right upper limb, including shoulder - Melanoma in situ of left upper limb, including shoulder
D03.71 - D03.72 Melanoma in situ of right lower limb, including hip - Melanoma in situ of left lower limb, including hip
D03.8 Melanoma in situ of other sites
D04.0 Carcinoma in situ of skin of lip
D04.111 - D04.112 Carcinoma in situ of skin of right upper eyelid, including canthus - Carcinoma in situ of skin of right lower eyelid, including canthus
D04.121 - D04.122 Carcinoma in situ of skin of left upper eyelid, including canthus - Carcinoma in situ of skin of left lower eyelid, including canthus
D04.21 - D04.22 Carcinoma in situ of skin of right ear and external auricular canal - Carcinoma in situ of skin of left ear and external auricular canal
D04.39 Carcinoma in situ of skin of other parts of face
D04.4 Carcinoma in situ of skin of scalp and neck
D04.5 Carcinoma in situ of skin of trunk
D04.61 - D04.62 Carcinoma in situ of skin of right upper limb, including shoulder - Carcinoma in situ of skin of left upper limb, including shoulder
D04.71 - D04.72 Carcinoma in situ of skin of right lower limb, including hip - Carcinoma in situ of skin of left lower limb, including hip
D04.8 Carcinoma in situ of skin of other sites
D05.01 - D05.02 Lobular carcinoma in situ of right breast - Lobular carcinoma in situ of left breast
D05.11 - D05.12 Intraductal carcinoma in situ of right breast - Intraductal carcinoma in situ of left breast
D05.81 - D05.82 Other specified type of carcinoma in situ of right breast - Other specified type of carcinoma in situ of left breast
D05.91 - D05.92 Unspecified type of carcinoma in situ of right breast - Unspecified type of carcinoma in situ of left breast
D06.0 - D06.7 Carcinoma in situ of endocervix - Carcinoma in situ of other parts of cervix
D07.0 - D07.2 Carcinoma in situ of endometrium - Carcinoma in situ of vagina
D07.39 Carcinoma in situ of other female genital organs
D07.4 - D07.5 Carcinoma in situ of penis - Carcinoma in situ of prostate
D07.61 - D07.69 Carcinoma in situ of scrotum - Carcinoma in situ of other male genital organs
D09.0 Carcinoma in situ of bladder
D09.19 Carcinoma in situ of other urinary organs
D09.21 - D09.22 Carcinoma in situ of right eye - Carcinoma in situ of left eye
D09.3 Carcinoma in situ of thyroid and other endocrine glands
D09.8 Carcinoma in situ of other specified sites
D37.01 - D37.02 Neoplasm of uncertain behavior of lip - Neoplasm of uncertain behavior of tongue
D37.030 - D37.039 Neoplasm of uncertain behavior of the parotid salivary glands - Neoplasm of uncertain behavior of the major salivary glands, unspecified
D37.04 - D37.05 Neoplasm of uncertain behavior of the minor salivary glands - Neoplasm of uncertain behavior of pharynx
D37.09 Neoplasm of uncertain behavior of other specified sites of the oral cavity
D37.1 - D37.8 Neoplasm of uncertain behavior of stomach - Neoplasm of uncertain behavior of other specified digestive organs
D38.0 - D38.5 Neoplasm of uncertain behavior of larynx - Neoplasm of uncertain behavior of other respiratory organs
D39.0 Neoplasm of uncertain behavior of uterus
D39.11 - D39.12 Neoplasm of uncertain behavior of right ovary - Neoplasm of uncertain behavior of left ovary
D39.2 Neoplasm of uncertain behavior of placenta
D39.8 Neoplasm of uncertain behavior of other specified female genital organs
D40.0 Neoplasm of uncertain behavior of prostate
D40.11 - D40.12 Neoplasm of uncertain behavior of right testis - Neoplasm of uncertain behavior of left testis
D41.11 - D41.22 Neoplasm of uncertain behavior of right renal pelvis - Neoplasm of uncertain behavior of left ureter
D41.3 - D41.8 Neoplasm of uncertain behavior of urethra - Neoplasm of uncertain behavior of other specified urinary organs
D42.0 - D42.1 Neoplasm of uncertain behavior of cerebral meninges - Neoplasm of uncertain behavior of spinal meninges
D43.0 - D43.8 Neoplasm of uncertain behavior of brain, supratentorial - Neoplasm of uncertain behavior of other specified parts of central nervous system
D44.0 Neoplasm of uncertain behavior of thyroid gland
D44.11 - D44.12 Neoplasm of uncertain behavior of right adrenal gland - Neoplasm of uncertain behavior of left adrenal gland
D44.2 - D44.7 Neoplasm of uncertain behavior of parathyroid gland - Neoplasm of uncertain behavior of aortic body and other paraganglia
D47.01 - D47.09 Cutaneous mastocytosis - Other mast cell neoplasms of uncertain behavior
D47.Z9 Other specified neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue
D48.0 - D48.5 Neoplasm of uncertain behavior of bone and articular cartilage - Neoplasm of uncertain behavior of skin
D48.61 - D48.62 Neoplasm of uncertain behavior of right breast - Neoplasm of uncertain behavior of left breast
D48.7 Neoplasm of uncertain behavior of other specified sites
D49.0 - D49.4 Neoplasm of unspecified behavior of digestive system - Neoplasm of unspecified behavior of bladder
D49.511 - D49.512 Neoplasm of unspecified behavior of right kidney - Neoplasm of unspecified behavior of left kidney
D49.59 Neoplasm of unspecified behavior of other genitourinary organ
D49.6 Neoplasm of unspecified behavior of brain
D49.7 Neoplasm of unspecified behavior of endocrine glands and other parts of nervous system
D49.81 Neoplasm of unspecified behavior of retina and choroid
D49.89 Neoplasm of unspecified behavior of other specified sites
Q85.01 - Q85.09 Neurofibromatosis, type 1 - Other neurofibromatosis

Group 4

(3 Codes)
Group 4 Paragraph

For PATIENTS WITH SYMPTOMATIC ANEMIA IN THE SETTING OF HIV DISEASE TREATED WITH ZIDOVUDINE

Dual diagnoses are expected; an ICD-10 code noting drug-induced aplastic anemia and an ICD-10 code noting the HIV infection or disease.

It is the provider’s responsibility to select ICD-10 codes carried out to the highest level of specificity.

The diagnosis codes listed below require the use of the EC modifier (ESA administered to treat anemia not due to anti-cancer radiotherapy or anti-cancer chemotherapy) when submitting claims for HCPCS code J0885 or Q5106. A JA or JB modifier is required.

The following dual ICD-10-CM codes support medical necessity and provide coverage for HCPCS code: J0885 or Q5106.

D61.1 Drug-induced aplastic anemia in conjunction with one of the following:

Group 4 Codes
CodeDescription
B20 Human immunodeficiency virus [HIV] disease
B97.35 Human immunodeficiency virus, type 2 [HIV 2] as the cause of diseases classified elsewhere
D61.1 Drug-induced aplastic anemia

Group 5

(13 Codes)
Group 5 Paragraph

For PATIENTS WITH SYMPTOMATIC ANEMIA IN THE SETTING OF MDS

An ICD-10 code noting the MDS must be reported.

It is the provider’s responsibility to select ICD-10 codes carried out to the highest level of specificity.

The diagnosis codes listed below require the use of the EC modifier (ESA administered to treat anemia not due to anti-cancer radiotherapy or anti-cancer chemotherapy) when submitting claims for HCPCS code J0881, J0885, or Q5106. A JA or JB modifier is required.

The following ICD-10-CM codes support medical necessity and provide coverage for HCPCS codes: J0881, J0885 or Q5106 when reasonable and necessary for the treatment of symptomatic anemia and/or RBC transfusion dependence. For MDS or Waldenstrom macroglobulinemia diagnosis codes noted below-the singular diagnosis code will suffice. For a rare myeloproliferative condition such as essential thrombocythemia, a secondary ICD-10 diagnosis code designating bone marrow disease impact with decreased RBC production must be present.

D47.3 Essential (hemorrhagic) thrombocythemia in conjunction with:  D75.81 Myelofibrosis

Group 5 Codes
CodeDescription
C88.0 Waldenstrom macroglobulinemia
D46.0 Refractory anemia without ring sideroblasts, so stated
D46.1 Refractory anemia with ring sideroblasts
D46.20 Refractory anemia with excess of blasts, unspecified
D46.21 Refractory anemia with excess of blasts 1
D46.22 Refractory anemia with excess of blasts 2
D46.A Refractory cytopenia with multilineage dysplasia
D46.B Refractory cytopenia with multilineage dysplasia and ring sideroblasts
D46.C Myelodysplastic syndrome with isolated del(5q) chromosomal abnormality
D46.Z Other myelodysplastic syndromes
D47.3 Essential (hemorrhagic) thrombocythemia
D75.81 Myelofibrosis
D75.84 Other platelet-activating anti-PF4 disorders

Group 6

(19 Codes)
Group 6 Paragraph

For PRESURGICAL ESA IN PATIENTS WITH PLANNED ELECTIVE MAJOR HIP OR KNEE SURGERY FOR REDUCTION OF ALLOGENEIC BLOOD TRANSFUSION

Three diagnoses are expected; an ICD-10 code noting type of anemia (D63.8) as primary diagnosis and Z01.818 as a secondary diagnosis along with one applicable osteoarthritis code.

It is the provider’s responsibility to select ICD-10 codes carried out to the highest level of specificity.

The diagnosis codes listed below require the use of the EC modifier (ESA administered to treat anemia not due to anti-cancer radiotherapy or anti-cancer chemotherapy) when submitting claims for HCPCS code J0885 or Q5106. A JA or JB modifier is required; a JB modifier would be consistent with the FDA indicated administration.

The following triad of ICD-10-CM codes support medical necessity and provide coverage for HCPCS code: J0885 or Q5106.

D63.8 Anemia in other chronic diseases classified elsewhere AND Z01.818 Encounter for other preprocedural examination in conjunction with one of the following:

Group 6 Codes
CodeDescription
D63.8 Anemia in other chronic diseases classified elsewhere
M16.0 Bilateral primary osteoarthritis of hip
M16.11 Unilateral primary osteoarthritis, right hip
M16.12 Unilateral primary osteoarthritis, left hip
M16.31 Unilateral osteoarthritis resulting from hip dysplasia, right hip
M16.32 Unilateral osteoarthritis resulting from hip dysplasia, left hip
M16.51 Unilateral post-traumatic osteoarthritis, right hip
M16.52 Unilateral post-traumatic osteoarthritis, left hip
M16.6 Other bilateral secondary osteoarthritis of hip
M16.7 Other unilateral secondary osteoarthritis of hip
M17.0 Bilateral primary osteoarthritis of knee
M17.11 Unilateral primary osteoarthritis, right knee
M17.12 Unilateral primary osteoarthritis, left knee
M17.2 Bilateral post-traumatic osteoarthritis of knee
M17.31 Unilateral post-traumatic osteoarthritis, right knee
M17.32 Unilateral post-traumatic osteoarthritis, left knee
M17.4 Other bilateral secondary osteoarthritis of knee
M17.5 Other unilateral secondary osteoarthritis of knee
Z01.818 Encounter for other preprocedural examination

ICD-10-CM Codes that DO NOT Support Medical Necessity

N/A

ICD-10-PCS Codes

N/A

Additional ICD-10 Information

N/A

Bill Type Codes

Contractors may specify Bill Types to help providers identify those Bill Types typically used to report this service. Absence of a Bill Type does not guarantee that the article does not apply to that Bill Type. Complete absence of all Bill Types indicates that coverage is not influenced by Bill Type and the article should be assumed to apply equally to all claims.

N/A

Revenue Codes

Contractors may specify Revenue Codes to help providers identify those Revenue Codes typically used to report this service. In most instances Revenue Codes are purely advisory. Unless specified in the article, services reported under other Revenue Codes are equally subject to this coverage determination. Complete absence of all Revenue Codes indicates that coverage is not influenced by Revenue Code and the article should be assumed to apply equally to all Revenue Codes.

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Other Coding Information

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Revision History Information

Revision History DateRevision History NumberRevision History Explanation
10/01/2022 R2

Under ICD-10-CM Codes that Support Medical Necessity Group 3: Codes the description was revised for C84.41 and C84.49. Under ICD-10-CM Codes that Support Medical Necessity Group 5: Codes added D75.84. This revision is due to the Annual ICD-10-CM Update and will become effective on 10/1/22.

07/24/2022 R1

Under Article Text added clarifying verbiage in multiple subsections regarding route of administration guidelines. Under ICD-10-CM Codes that Support Medical Necessity – all Group Paragraphs added clarifying verbiage regarding route of administration guidelines. Typographical errors were corrected throughout the LCD.

Associated Documents

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LCDs
L39237 - Erythropoiesis Stimulating Agents
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