D59.5 — Paroxysmal nocturnal hemoglobinuria [Marchiafava-Micheli]
ICD-10-CM
Prior Authorization
No Prior Auth Required
Code is covered without prior authorization (high confidence)
Documentation Required
- Document meningococcal immunization at least 2 weeks prior to the first eculizumab dose or a clinical justification that delaying eculizumab posed greater risk than meningococcal infection.
- For PNH initiation, document flow cytometry confirming PNH and either evidence of clinically significant hemolysis or a documented history of a major adverse vascular event (MAVE) from thromboembolism.
- For aHUS initial trial, document absence of signs of STEC-HUS and that TTP was ruled out (e.g., normal ADAMTS13 activity and no inhibitor), or document a plasma exchange trial that failed to produce clinical improvement.
- If continuing eculizumab after the aHUS initial trial, document clinical improvement such as increased platelet count or laboratory evidence of reduced hemolysis.
Key Coverage Criteria
- Eculizumab is covered for treatment of paroxysmal nocturnal hemoglobinuria (PNH) when flow cytometry confirms PNH and there is either evidence of clinically significant hemolysis or a documented history of a major adverse vascular event (MAVE) from thromboembolism.
- Eculizumab is covered for treatment of atypical hemolytic uremic syndrome (aHUS) as an initial trial (generally 6–12 weeks) when the individual has no signs of STEC-HUS and thrombotic thrombocytopenic purpura (TTP) has been ruled out or a trial of plasma exchange did not produce clinical improvement.
- Eculizumab is covered for biopsy-proven dense deposit disease when elevated serum sC5b-9 (sMAC) levels are documented.
- Eculizumab may be covered for other label and off-label indications supported by recognized compendia or the FDA label when dose and frequency conform to FDA labeling or recognized compendia guidance.
- Coverage and medical necessity determinations for flow cytometry are governed by Local Coverage Determination L34513.
- Flow cytometry may be used to analyze cellular components of body fluids and lymphoreticular tissues, including peripheral blood, cerebrospinal fluid (CSF), bone marrow, lymph nodes, tonsil, spleen, and fine needle aspirate material.
13 Active Policies
L33394 — Drugs and Biologicals, Coverage of, for Label and Off-Label Uses
J06
A54548 — Billing and Coding: Eculizumab, and Biosimilars: EPYSQLI-eculizumab-aagh, and BKEMV-eculizumab-aeeb
J06
AETNA-CPB-0351 — Flow Cytometry, Ektacytometry, DNA Ploidy, and S-phase Fraction
AETNA-CPB-0634 — Non-myeloablative Hematopoietic Cell Transplantation (Mini-Allograft / Reduced Intensity Conditioning Transplant)
Related Codes
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