Anthem Blue Cross California Istodax (romidepsin) Form

Overview Coding References Clinical criteria Document history Overview This document addresses the use of romidepsin, an intravenously administered histone deacetylase (HDAC) inhibitor. HDAC inhibitors are useful as antineoplastic agents as they cause accumulation of acetylated histones, inducing cell cycle arrest and/or apoptosis of some transformed cells. It is used to treat a certain subset of non- hodgkins lymphoma (NHL) known as T-Cell lymphomas. T-cell lymphomas account for approximately 15% of all non- Hodgkin lymphoma in the United States. NHLs are a broad and diverse group of malignancies affecting both B- and T-lymphocytes. T-Cell Lymphomas can broadly be classified as cutaneous or non-cutaneous. Romidepsin is currently FDA approved for cutaneous T-cell lymphoma in patients that have received at least one prior therapy. The FDA recently revoked an additional indication for relapsed peripheral t-cell lymphoma which was under accelerated approval. The National Comprehensive Cancer Network® (NCCN) provides additional recommendations with a category 2A level of evidence for the use of romidepsin as primary treatment for Mycosis Fungoides/Sezary Syndrome and for the cutaneous form of anaplastic large cell lymphoma (ALCL), known as primary cutaneous ALCL. While NCCN includes romidepsin as a treatment option for other types of T-cell lymphomas, such as peripheral t-cell lymphoma (PTCL), extranodal NK/T-Cell lymphoma, nasal type (NKTL), and hepatosplenic gamma-delta T-Cell Lymphoma (HGTL), the accelerated approval for romidepsin in PTCL was removed in 2021. The accelerated approval status for romidepsin for the treatment of relapsed/refractory PTCL was withdrawn following the results of the confirmatory phase III trial, which failed to meet the primary endpoint of improved PFS for romidepsin + CHOP in patients with previously untreated PTCL (421 patients randomized to receive romidepsin + CHOP or CHOP). After a median follow-up of 28 months the addition of romidepsin to CHOP did not result in any statistically significant improvement in ORR, PFS, or OS but increased the frequency of grade ≥3 adverse events. While the panel acknowledged the change in the regulatory status of romidepsin, the consensus of the panel was to continue the listing of romidepsin as an important option for relapsed or refractory PTCL based on the results of the earlier phase II study and subsequent studies in which romidepsin resulted in durable responses across all three subtypes of PTCL (Anaplastic large cell lymphoma (ALCL), Anaplastic lymphoma kinase (ALK)-negative, PTCL-not otherwise specified (NOS), and Angioimmunoblastic T-cell lymphoma (AITL)). Definitions and Measures Mycosis fungoides/ Sézary Syndrome (MF/SS): Cutaneous T-cell Lymphomas (CTCLs) are a group of NHLs of mature T-cells that primarily present in the skin, and at times progress to involve lymph nodes, blood, and visceral organs. MF is the most common subtype with primary cutaneous involvement and SS is an erythrodermic, leukemic variant of CTCL that is characterized by significant blood involvement and lymphadenopathy. Refractory Disease: Illness or disease that does not respond to treatment. Relapse or recurrence: After a period of improvement, during which time a disease (for example, cancer) could not be detected, the return of signs and symptoms of illness or disease. For cancer, it may come back to the same place as the original (primary) tumor or to another place in the body. Clinical Criteria 1 When a drug is being reviewed for coverage under a member’s medical benefit plan or is otherwise subject to clinical review (including prior authorization), the following criteria will be used to determine whether the drug meets any applicable medical necessity requirements for the intended/prescribed purpose. Romidepsin Requests for romidepsin may be approved if the following criteria are met: I. Individual has a diagnosis of cutaneous T-cell lymphoma; AND A. Individual is using for relapsed or refractory disease following at least one prior systemic therapy; OR II. OR III. OR IV. Individual has a diagnosis of Primary cutaneous anaplastic large cell lymphoma (ALCL) with multifocal lesions; AND A. B. Using romidepsin as monotherapy; Individual has relapsed or refractory disease; AND Individual has a diagnosis of cutaneous ALCL with regional node (N1); AND Individual has relapsed or refractory disease; AND A. B. Using romidepsin as monotherapy; Individual has a diagnosis of Mycosis Fungoides or Sézary Syndrome (NCCN 2A); Requests for romidepsin may not be approved when the above criteria are not met and for all other indications. Coding The following codes for treatments and procedures applicable to this document are included below for informational purposes. Inclusion or exclusion of a procedure, diagnosis or device code(s) does not constitute or imply member coverage or provider reimbursement policy. Please refer to the member's contract benefits in effect at the time of service to determine coverage or non-coverage of these services as it applies to an individual member. HCPCS J9318 J9319 ICD-10 Diagnosis C84.00 C84.01 C84.02 C84.03 C84.04 C84.05 C84.06 C84.07 C84.08 C84.09 C84.10 C84.11 C84.12 Injection, romidepsin, nonlyophilized, 0.1 mg Injection, romidepsin, lyophilized, 0.1 mg Mycosis fungoides, unspecified site Mycosis fungoides, lymph nodes of head, face, and neck Mycosis fungoides, intrathoracic lymph nodes Mycosis fungoides, intra-abdominal lymph nodes Mycosis fungoides, lymph nodes of axilla and upper limb Mycosis fungoides, lymph nodes of inguinal region and lower limb Mycosis fungoides, intrapelvic lymph nodes Mycosis fungoides, spleen Mycosis fungoides, lymph nodes of multiple sites Mycosis fungoides, extranodal and solid organ sites Sezary disease, unspecified site Sezary disease, lymph nodes of head, face, and neck Sezary disease, intrathoracic lymph nodes 2 C84.13 C84.14 C84.15 C84.16 C84.17 C84.18 C84.19 C84.40 C84.41 C84.42 C84.43 C84.44 C84.45 C84.46 C84.47 C84.48 C84.49 C84.60 C84.61 C84.62 C84.63 C84.64 C84.65 C84.66 C84.67 C84.68 C84.69 C84.70 C84.71 C84.72 C84.73 C84.74 C84.75 C84.76 C84.77 C84.78 C84.79 C84.7A Sezary disease, intra-abdominal lymph nodes Sezary disease, lymph nodes of axilla and upper limb Sezary disease, lymph nodes of inguinal region and lower limb Sezary disease, intrapelvic lymph nodes Sezary disease, spleen Sezary disease, lymph nodes of multiple sites Sezary disease, extranodal and solid organ sites Peripheral T-cell lymphoma, not elsewhere classified, unspecified site Peripheral T-cell lymphoma, not elsewhere classified, lymph nodes of head, face, and neck Peripheral T-cell lymphoma, not elsewhere classified, intrathoracic lymph nodes Peripheral T-cell lymphoma, not elsewhere classified, intra-abdominal lymph nodes Peripheral T-cell lymphoma, not elsewhere classified, lymph nodes of axilla and upper limb Peripheral T-cell lymphoma, not elsewhere classified, lymph nodes of inguinal region and lower limb Peripheral T-cell lymphoma, not elsewhere classified, intrapelvic lymph nodes Peripheral T-cell lymphoma, not elsewhere classified, spleen Peripheral T-cell lymphoma, not elsewhere classified, lymph nodes of multiple sites Peripheral T-cell lymphoma, not elsewhere classified, extranodal and solid organ sites Anaplastic large cell lymphoma, ALK-positive, unspecified site Anaplastic large cell lymphoma, ALK-positive, lymph nodes of head, face, and neck Anaplastic large cell lymphoma, ALK-positive, intrathoracic lymph nodes Anaplastic large cell lymphoma, ALK-positive, intra-abdominal lymph nodes Anaplastic large cell lymphoma, ALK-positive, lymph nodes of axilla and upper limb Anaplastic large cell lymphoma, ALK-positive, lymph nodes of inguinal region and lower limb Anaplastic large cell lymphoma, ALK-positive, intrapelvic lymph nodes Anaplastic large cell lymphoma, ALK-positive, spleen Anaplastic large cell lymphoma, ALK-positive, lymph nodes of multiple sites Anaplastic large cell lymphoma, ALK-positive, extranodal and solid organ sites Anaplastic large cell lymphoma, ALK-negative, unspecified site Anaplastic large cell lymphoma, ALK-negative, lymph nodes of head, face, and neck Anaplastic large cell lymphoma, ALK-negative, intrathoracic lymph nodes Anaplastic large cell lymphoma, ALK-negative, intra-abdominal lymph nodes Anaplastic large cell lymphoma, ALK-negative, lymph nodes of axilla and upper limb Anaplastic large cell lymphoma, ALK-negative, lymph nodes of inguinal region and lower limb Anaplastic large cell lymphoma, ALK-negative, intrapelvic lymph nodes Anaplastic large cell lymphoma, ALK-negative, spleen Anaplastic large cell lymphoma, ALK-negative, lymph nodes of multiple sites Anaplastic large cell lymphoma, ALK-negative, extranodal and solid organ sites Anaplastic large cell lymphoma, ALK-negative, breast 3 C84.A0-C84.A9 Cutaneous T-cell lymphoma, unspecified Extranodal NK/T-cell lymphoma, nasal type Hepatosplenic T-cell lymphoma Enteropathy-type (intestinal) T-cell lymphoma Subcutaneous panniculitis-like T-cell lymphoma Angioimmunoblastic T-cell lymphoma Primary cutaneous CD30-positive T-cell proliferations Personal history of non-Hodgkin lymphomas C86.0 C86.1 C86.2 C86.3 C86.5 C86.6 Z85.72 Document History Reviewed: 08/18/2023