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Superseded Local Coverage Determination (LCD)

Hemophilia Clotting Factors

L33684

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To see the currently-in-effect version of this document, go to the section.

Contractor Information

LCD Information

Document Information

LCD ID
L33684
LCD Title
Hemophilia Clotting Factors
Proposed LCD in Comment Period
N/A
Source Proposed LCD
N/A
Original Effective Date
For services performed on or after 10/01/2015
Revision Effective Date
For services performed on or after 07/01/2019
Revision Ending Date
N/A
Retirement Date
N/A
Notice Period Start Date
N/A
Notice Period End Date
N/A
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Fee schedules, relative value units, conversion factors and/or related components are not assigned by the AMA, are not part of CPT, and the AMA is not recommending their use. The AMA does not directly or indirectly practice medicine or dispense medical services. The AMA assumes no liability for data contained or not contained herein.

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Copyright © 2022, the American Hospital Association, Chicago, Illinois. Reproduced with permission. No portion of the American Hospital Association (AHA) copyrighted materials contained within this publication may be copied without the express written consent of the AHA. AHA copyrighted materials including the UB‐04 codes and descriptions may not be removed, copied, or utilized within any software, product, service, solution or derivative work without the written consent of the AHA. If an entity wishes to utilize any AHA materials, please contact the AHA at 312‐893‐6816.

Making copies or utilizing the content of the UB‐04 Manual, including the codes and/or descriptions, for internal purposes, resale and/or to be used in any product or publication; creating any modified or derivative work of the UB‐04 Manual and/or codes and descriptions; and/or making any commercial use of UB‐04 Manual or any portion thereof, including the codes and/or descriptions, is only authorized with an express license from the American Hospital Association. The American Hospital Association (the "AHA") has not reviewed, and is not responsible for, the completeness or accuracy of any information contained in this material, nor was the AHA or any of its affiliates, involved in the preparation of this material, or the analysis of information provided in the material. The views and/or positions presented in the material do not necessarily represent the views of the AHA. CMS and its products and services are not endorsed by the AHA or any of its affiliates.

CMS National Coverage Policy

This LCD supplements but does not replace, modify or supersede existing Medicare applicable National Coverage Determinations (NCDs) or payment policy rules and regulations for Hemophilia Clotting Factors. Federal statute and subsequent Medicare regulations regarding provision and payment for medical services are lengthy. They are not repeated in this LCD. Neither Medicare payment policy rules nor this LCD replace, modify or supersede applicable state statutes regarding medical practice or other health practice professions acts, definitions and/or scopes of practice. All providers who report services for Medicare payment must fully understand and follow all existing laws, regulations and rules for Medicare payment for Hemophilia Clotting Factors and must properly submit only valid claims for them. Please review and understand them and apply the medical necessity provisions in the policy within the context of the manual rules. Relevant CMS manual instructions and policies may be found in the following Internet-Only Manuals (IOMs) published on the CMS Web site.

Internet Only Manual (IOM) Citations:

  • CMS IOM Publication 100-02, Medicare Benefit Policy Manual,
    • Chapter 6, Section 30 Drugs and Biologicals
    • Chapter 8, Section 70 Medical and Other Health Services Furnished to SNF Patients
    • Chapter 15, Section 50.5.5 Hemophilia Clotting Factors
  • CMS IOM Publication 100-04, Medicare Claims Processing Manual, Chapter 3, Section 20.7.3 Payment for Blood Clotting Factor Administered to Hemophilia Inpatients
  • CMS IOM Publication 100-08, Medicare Program Integrity Manual, Chapter 13, Section 13.5.4 Reasonable and Necessary Provision in an LCD

Social Security Act (Title XVIII) Standard References:

  • Title XVIII of the Social Security Act, Section 1862(a)(1)(A) states that no Medicare payment shall be made for items or services which are not reasonable and necessary for the diagnosis or treatment of illness or injury.
  • Title XVIII of the Social Security Act, Section 1862(a)(7). This section excludes routine physical examinations.
  • Title XVIII of the Social Security Act, Section 1833(e) states that no payment shall be made to any provider for any claim that lacks the necessary information to process the claim.

Coverage Guidance

Coverage Indications, Limitations, and/or Medical Necessity

Hemophilia is a hereditary blood disease characterized by greatly prolonged coagulation time. The blood fails to clot and abnormal bleeding occurs. It is a sex-linked hereditary trait transmitted by normal heterozygous females who carry the recessive gene. It occurs almost exclusively in males. Hemophilia encompasses Factor VIII deficiency (classic hemophilia, hemophilia A), Factor IX deficiency (hemophilia B, Christmas disease, plasma thromboplastin component), and von Willebrand’s disease. Approximately 80% of those with hemophilia have type A and both are associated with recurrent, spontaneous, and traumatic hemarthrosis.

In rare cases, hemophilia can develop after birth, which is called acquired hemophilia caused by the development of antibodies (immune system proteins) directed against the body’s own VIII or IX blood clotting factors. Unlike inherited hemophilia, acquired hemophilia A is not a genetic disorder and affects both males and females. The development of acquired hemophilia A has been related to other medical conditions or health states, such as pregnancy, cancer, or the use of certain medications. However, in about half of the cases, no underlying cause can be found.

The frequency and severity of hemorrhagic events induced by hemophilia are related to the amount of coagulation factor in the blood. Those with mild hemophilia (defined as having from 5% to 40% of normal coagulation factor activity) experience complications only after having undergone surgery or experiencing a major physical trauma. Those with moderate hemophilia (from 1% to 5% of coagulation factor activity) experience some spontaneous hemorrhage but normally exhibit bleeding provoked by trauma. Those with severe hemophilia (less than 1% of coagulation factor activity) exhibit spontaneous hemarthrosis and bleeding. Treatment for these patients is dependent on the severity of the disease and may include the administration of blood clotting factors such as Factor VIII, Factor IX, Factor VIIa and, Anti-inhibitors to control the bleeding.

Covered Indications

Coverage is provided of self-administered blood clotting factors for hemophilia patients who are competent to use such factors to control bleeding without medical supervision. Coverage of blood clotting factors for the following conditions is provided:

  • Factor VIII deficiency (classic hemophilia, hemophilia A).
  • Factor IX deficiency (hemophilia B, Christmas disease, plasma thromboplastin component).
  • von Willebrand’s disease.

Anti-inhibitor coagulant complex (AICC) is a drug used to treat hemophilia in patients with Factor VIII inhibitor antibodies. AICC has been shown to be safe and effective and is covered when furnished to patients with hemophilia A and inhibitor antibodies to Factor VIII who have major bleeding episodes and who fail to respond to other less expensive therapies.

Antihemophilic factor is usually indicated for hemophilia when a bleeding episode arises (demand treatment) or when bleeding is anticipated or likely (prophylactic treatment). Primary prophylactic therapy may be indicated for patients with severe hemophilia A or B who have less than 1 percent of normal factor (less than 0.01 IU/mL (National Hemophilia Foundation, 2007). Primary prophylactic therapy should be instituted early, prior to the onset of frequent bleeding, with the aim of keeping the trough factor or Factor VIII or Factor IX level above 1 percent between doses (National Hemophilia Foundation, 2007). In some cases, continuous prophylactic therapy may be indicated in persons with hemophilia A or hemophilia B that is not severe (i.e., hemophiliacs with more than 1 percent of normal factor levels) who have repeated episodes of spontaneous bleeding.

Limitations

As published in the CMS IOM Publication 100-08, Medicare Program Integrity Manual, Chapter 13, Section 13.5.4, an item or service may be covered by a contractor LCD if it is reasonable and necessary under the Social Security Act Section 1862 (a)(1)(A). Contractors shall determine and describe the circumstances under which the item or service is considered reasonable and necessary.
Summary of Evidence

N/A
Analysis of Evidence (Rationale for Determination)

N/A

General Information

Associated Information

Documentation Requirements

Please refer to the Local Coverage Article: Billing and Coding: Hemophilia Clotting Factors (A56482) for documentation requirements that apply to the reasonable and necessary provisions outlined in this LCD.



Utilization Guidelines

Please refer to the Local Coverage Article: Billing and Coding: Hemophilia Clotting Factors (A56482) for utilization guidelines that apply to the reasonable and necessary provisions outlined in this LCD.
Sources of Information
  1. First Coast Service Options, Inc. reference LCD number(s) – L28884, L29187, L29345
  2. Business Wire—The WSJ (March 28, 2014) FDA Approves Biogen Idec's ALPROLIX(TM), the First Hemophilia B Therapy to Reduce Bleeding Episodes with Prophylactic Infusions Starting at Least a Week Apart
  3. Food and Drug Administration (FDA) website—November 16, 2017 Label Prescribing Information – HEMLIBRA® (emicizumab-kxwh) injection.
  4. Food and Drug Administration (FDA) website—May 31, 2017 Label Prescribing Information – REBINYN® (Coagulation Factor IX (Recombinant), GlycoPEGylated) injection.
  5. Food and Drug Administration (FDA) website— March 4, 2016 Label Prescribing Information – IDELVION
  6. Food and Drug Administration (FDA) website— October 23, 2014 Approval Letter – OBIZUR
  7. Novitas LCD for Hemophilia Factor Products (L33658)
Bibliography
  1. Mahlangu, J, Powel JS, Pasi KJ, Ragni MV, Phase 3 Study of Recombinant Factor VII Fc Fusion Protein in Hemophilia A. Blood. 2014; 123:317-325.
  2. National Hemophilia Foundation: MASAC Recommendation Concerning Prophylaxis, November 4, 2007.
  3. Peters RT, Toby G, Lu Q. Biochemical and Functional Characterization of a Recombinant Monomeric Factor VIII-Fc Fusion Protein. J Thromb Hemost. 2013; 11(1): 132-41.
  4. Roberts HR, Monroe DM, Escobar, MA. Current Concepts of Hemostasis. Anesthesiology. 2004; 100:722-30. This source used to provide information about the role of blood clotting factors in hemostasis.
  5. Saunders WB. Goldman: Cecil Textbook of Medicine. Hereditary coagulation deficiencies; 2004:1069-75. This source used to gain textbook knowledge of hemophilia and the physiological implications of the disease.
  6. Shapiro A. Inhibitor treatment: State of the art. Disease-A-Month 2003:49. This source provides information on the efficacy of the administration of blood clotting factors to assist in preventing and reducing bleeding episodes.

Revision History Information

Revision History DateRevision History NumberRevision History ExplanationReasons for Change
07/01/2019 R20

12/06/2019: The content in the LCD was revised to be consistent with the new format supported by CMS IOM Publication 100-08, Medicare Program Integrity Manual, Chapter 13, Section 13.5.1.
  • Other
07/01/2019 R19

Revision Number: 9
Publication: June 2019 Connection
LCR A/B2019-033

Explanation of Revision: Based on review of the LCD, typographical and formatting errors were corrected. The effective date of this revision is based on date of service.

07/01/2019:  At this time 21st Century Cures Act will apply to new and revised LCDs that restrict coverage which requires comment and notice. This revision is not a restriction to the coverage determination and therefore not all the fields included on the LCD are applicable as noted in this LCD.
  • Typographical Error
04/01/2019 R18

Revision Number: 8
Publication: April 2019 Connection
LCR A/B2019-028

Explanation of Revision: Based on CR 10901, the LCD was revised to remove all billing and coding and all language not related to reasonable and necessary provisions (“Bill Type Codes,” “Revenue Codes,” “CPT/HCPCS Codes,” “ICD-10 Codes that Support Medical Necessity,” “Documentation Requirements,” and “Utilization Guidelines” sections of the LCD) and place them into a newly created billing and coding article. Also, the LCD was revised to update Internet-Only Manual (IOM) references in the “CMS National Coverage Policy” section of the LCD. The effective date of this LCD revision is for claims processed on or after January 8, 2019, for dates of service on or after October 3, 2018. Additionally, based on CRs 11192, 11216, and 11232, the LCD billing and coding article was revised to add HCPCS codes C9141 and J7199 (Injection, factor viii, [antihemophilic factor, recombinant], pegylated-aucl [Jivi], 1 i.u). The effective date of this LCD revision is for dates of service on or after April 1, 2019.

04/01/2019: At this time 21st Century Cures Act will apply to new and revised LCDs that restrict coverage which requires comment and notice. This revision is not a restriction to the coverage determination and therefore not all the fields included on the LCD are applicable as noted in this LCD.
  • Other (Revisions due to Change Requests (CR) 11192, 11216, 11232, and 10901)
01/01/2019 R17

Revision Number: 7
Publication: December 2018 Connection
LCR A/B2019-001

Explanation of Revision: Annual 2019 HCPCS Update. The LCD was revised to add HCPCS codes J7170 and J7203 and corresponding ICD-10-CM diagnosis codes D66 and D67, respectively, in the “CPT/HCPCS Codes” and “ICD-10 Codes that Support Medical Necessity” sections of the LCD. Also, the “Sources of information” section of the LCD was updated. In addition, grammatical errors in the LCD were corrected. The effective date of this revision is based on date of service.

01/01/2019: At this time 21st Century Cures Act will apply to new and revised LCDs that restrict coverage which requires comment and notice. This revision is not a restriction to the coverage determination and therefore not all the fields included on the LCD are applicable as noted in this LCD.
  • Revisions Due To CPT/HCPCS Code Changes
01/01/2018 R16

Revision Number: 6

Publication: December 2017 Connection

LCR A/B2018-001

Explanation of Revision: Explanation of Revision: Annual 2018 HCPCS Update. Removed HCPCS code J7199 (Afstyla) and deleted HCPCS code C9140 and replaced with HCPCS code J7210. In addition, added HCPCS code J7211. In addition, based on CR 10385 (January 2018 Integrated Outpatient Code Editior [I/OCE]), HCPCS code J7191 was changed from “Part A and Part B” to “Part B only” in the “CPT/HCPCS codes” section of the LCD as this code has a Part A status indicator “E2” (Not paid by Medicare when submitted on outpatient claims). Also, HCPCS code J7191 was removed from “Group 1 Paragraph:” under “ICD-10 Codes that Support Medical Necessity” section of the LCD and put in its own group. The effective date of this revision is based on date of service.

01/01/2018:  At this time 21st Century Cures Act will apply to new and revised LCDs that restrict coverage which requires comment and notice.  This revision is not a restriction to the coverage determination and therefore not all the fields included on the LCD are applicable as noted in this policy.
  • Revisions Due To CPT/HCPCS Code Changes
01/01/2017 R15 Revision Number: 5
Publication: December 2016 Connection
LCR A/B2017-001

Explanation of Revision: Annual 2017 HCPCS Update. The LCD was revised to delete HCPCS codes C9137, C9138, and C9139. In addition, HCPCS codes C9140, J7202, J7207, J7209, J7179, and J7199 (Afstyla) were added. Also, HCPCS code J7199 was removed for Idelvion, Nuwiq, and Adynovate. Also, HCPCS code J7182 was changed from Part B only to Part A and B. The effective date of this revision is based on date of service.
  • Other (Correction HCPCS code J7182 was changed from Part B only to Part A and B.)
01/01/2017 R14 Revision Number: 5
Publication: December 2016 Connection
LCR A/B2017-001

Explanation of Revision: Annual 2017 HCPCS Update. The LCD was revised to delete HCPCS codes C9137, C9138, and C9139. In addition, HCPCS codes C9140, J7202, J7207, J7209, J7179, and J7199 (Afstyla) were added. Also, HCPCS code J7199 was removed for Idelvion, Nuwiq, and Adynovate. The effective date of this revision is based on date of service.
  • Revisions Due To CPT/HCPCS Code Changes
10/01/2016 R13 Revision Number: 4
Publication: October 2016 Connection
LCR A/B2016-101

Explanation of Revision: Based on the CMS October 2016 Updates, CR 9768 (Hospital OPPS), CR 9773 (ASC Payment System), and CR 9754 (I/OCE), HCPCS code C9139 replaced HCPCS code C9399 in the “CPT/HCPCS Codes” and “ICD-10 Codes that Support Medical Necessity” sections of the LCD. The effective date of this revision is based on date of service.
  • Other
07/11/2016 R12 7/14/16 - added clarification in paragraph sections of "CPT/HCPCS" code groups 8 and 9 for unlisted codes in groups 8 and 9.
  • Reconsideration Request
07/11/2016 R11 Revision Number: 3
Publication: July 2016 Connection
LCR A/B2016-076

Explanation of Revision: Based on a reconsideration request and FDA approval, HCPCS codes J7199 (Idelvion) and C9399 (Idelvion) were added to the “CPT/HCPCS Codes” section of the LCD, and diagnosis code D67 was added to “ICD-10 Codes that Support Medical Necessity” section of the LCD. The “Sources of Information and Basis for Decision” section was also updated. The effective date of this revision is for claims processed on or after 07/11/2016, for dates of service on or after 3/4/2016.
  • Revisions Due To ICD-10-CM Code Changes
04/01/2016 R10 04/26/16 CPT codes C9137 and C9138 were removed from the group six (6) paragraph section to the group six (6) code section.
  • Other
04/01/2016 R9 Revision Number: 2
Publication: April 2016 Connection
LCR A/B2016-060

Explanation of Revision: Based on CR 9549 (April 2016 Update of the Hospital OPPS) and CR 9557 (April 2016 Update of the ASC Payment System), HCPCS codes C9137 and C9138 were added to the “CPT/HCPCS Codes” and “ICD-10 Codes that Support Medical Necessity” sections of the LCD. In addition, HCPCS code J7199 (Adynovate) and J7199 (Nuwiq) were added to the “CPT/HCPCS Codes” and “ICD-10 Codes that Support Medical Necessity” sections of the LCD. The effective date of this revision is based on date of service.
  • Revisions Due To CPT/HCPCS Code Changes
  • Revisions Due To ICD-10-CM Code Changes
01/01/2016 R8 The CPT code sections and ICD-10 diagnoses sections have been revised for further clarity, in regards to coverage.
  • Public Education/Guidance
01/01/2016 R7 Typographical error corrected.
  • Typographical Error
01/01/2016 R6 Revision Number: 1
Publication: December 2015 Connection
LCR A/B2016-009

Explanation of Revision: Annual 2016 HCPCS Update. HCPCS code Q9975 was deleted and replaced with HCPCS code J7205 and C9399/J7199 were removed and replaced with HCPCS code J7188. The effective date of this revision is based on date of service,
  • Public Education/Guidance
10/01/2015 R5 08/24/2015 - - The language and/or ICD-10-CM diagnoses were updated to be consistent with the current ICD-9-CM LCD’s language and coding.
  • Provider Education/Guidance
  • Public Education/Guidance
10/01/2015 R4 04/20/2015 – The language and/or ICD-10-CM diagnoses were updated to be consistent with current LCD language and ICD-9-CM coding.
  • Provider Education/Guidance
10/01/2015 R3 3/13/2015: The language and/or ICD-10-CM diagnoses were updated to be consistent with the current ICD-9-CM LCD’s language and coding.
  • Revisions Due To CPT/HCPCS Code Changes
10/01/2015 R2 8/22/2014 - The language and/or ICD-10-CM diagnoses were updated to be consistent with the current ICD-9-CM LCD’s language and coding.
  • Revisions Due To ICD-10-CM Code Changes
10/01/2015 R1 06/29/2014 - As a result of ICD-10 CM updates the following CPT/HCPCS code C9133 either the short description and/or the long description was changed.
  • Revisions Due To ICD-10-CM Code Changes

Associated Documents

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Related National Coverage Documents
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Updated On Effective Dates Status
10/14/2022 07/01/2019 - 10/01/2022 Retired View
12/08/2019 07/01/2019 - N/A Superseded You are here
06/21/2019 07/01/2019 - N/A Superseded View
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