Subject: Jakafi 1999-A SGM P2024a
Policy:
I. INDICATIONS
The indications below including FDA-approved indications and compendial uses are
considered a covered benefit provided that all the approval criteria are met and the member
has no exclusions to the prescribed therapy.
A. FDA-Approved Indications
1. Jakafi is indicated for treatment of intermediate or high-risk myelofibrosis (MF), including
primary MF, post-polycythemia vera MF and post-essential thrombocythemia MF in
adults.
2. Jakafi is indicated for treatment of polycythemia vera (PV) in adults who have had an
inadequate response to or are intolerant of hydroxyurea.
3. Jakafi is indicated for treatment of steroid-refractory acute graft-versus-host disease
(aGVHD) in adult and pediatric patients 12 years and older.
4. Jakafi is indicated for treatment of chronic graft-versus-host disease (cGVHD) after
failure of one or two lines of systemic therapy in adult and pediatric patients 12 years
and older.
B. Compendial Uses
1. Symptomatic lower risk myelofibrosis
2. Myelofibrosis-associated anemia
3. Accelerated/blast phase myeloproliferative neoplasms
4. Polycythemia vera in patients with inadequate response or loss of response to interferon
therapy
5. Polycythemia vera in patients with high-risk disease
6. Philadelphia chromosome (Ph-like) B-cell Acute Lymphoblastic Leukemia
(ALL)/Lymphoblastic lymphoma (LL)
7. Chronic myelomonocytic leukemia (CMML)-2
8. T-cell lymphomas - T-cell large granular lymphocytic leukemia and T-cell prolymphocytic
leukemia
9. BCR-ABL negative atypical chronic myeloid leukemia (aCML) /
Myelodysplastic/Myeloproliferative Neoplasms (MDS/MPN) with neutrophilia
10. Essential Thrombocythemia
11. Myeloid/lymphoid neoplasms with eosinophilia and JAK2 rearrangement in blast phase
or chronic phase
12. CAR T-cell-related toxicities - Cytokine release syndrome (CRS)
13. Immune checkpoint inhibitor-related toxicities - concomitant myositis and myocarditis
All other indications are considered experimental/investigational and not medically necessary.
II. DOCUMENTATION
Submission of the following information is necessary to initiate the prior authorization review:
A. For Ph-like B-cell acute lymphoblastic leukemia/lymphoblastic lymphoma (LL), medical
record documentation confirming either a cytokine receptor-like factor 2 (CRLF2) mutation
or a mutation associated with activation of the Janus kinase/signal transducers and
activators of transcription (JAK/STAT) pathway.
B. For myeloid and/or lymphoid neoplasms with eosinophilia: Testing or analysis confirming
JAK2 rearrangement
III. CRITERIA FOR INITIAL APPROVAL
A. Myelofibrosis
Authorization of 12 months may be granted for the treatment of myelofibrosis.
B. Accelerated/Blast Phase Myeloproliferative Neoplasms
Authorization of 12 months may be granted for the treatment of accelerated phase or blast
phase myeloproliferative neoplasms when used as a single agent or in combination with
azacitidine or decitabine.
C. Polycythemia Vera
Authorization of 12 months may be granted for the treatment of polycythemia vera when any
of the following criteria are met:
1. Member has had an inadequate response or intolerance to hydroxyurea or peginterferon
alfa-2a
2. Member has high risk disease
D. Acute Graft-versus-Host Disease (aGVHD) or Chronic Graft-versus-Host Disease
(cGVHD)
Authorization of 12 months may be granted for the treatment of graft-vs-host disease when
any of the following criteria are met:
1. Member has steroid-refractory acute GVHD
2. Member has chronic GVHD and has failed at least one prior line of systemic therapy
E. Acute Lymphoblastic Leukemia (ALL)/Lymphoblastic Lymphoma (LL)
Authorization of 12 months may be granted for the treatment of Ph-like B-cell acute
lymphoblastic leukemia/lymphoblastic lymphoma for members with either a cytokine
receptor-like factor 2 (CRLF2) mutation or a mutation associated with activation of the
Janus kinase/signal transducers and activators of transcription (JAK/STAT) pathway.
F. Chronic Myelomonocytic Leukemia (CMML)-2
Authorization of 12 months may be granted for the treatment of chronic myelomonocytic
leukemia (CMML)-2 in combination with a hypomethylating agent.
G. T-Cell Large Granular Lymphocytic Leukemia or T-Cell Prolymphocytic Leukemia
Authorization of 12 months may be granted for the subsequent treatment of T-cell large
granular lymphocytic leukemia or symptomatic T-cell prolymphocytic leukemia, as a single
agent.
H. Atypical Chronic Myeloid Leukemia (aCML) / Myelodysplastic/Myeloproliferative
Neoplasms (MDS/MPN) with Neutrophilia
Authorization of 12 months may be granted for the treatment of BCR-ABL negative aCML or
MDS/MPN with neutrophilia as a single agent or in combination with a hypomethylating
agent.
I. Essential Thrombocythemia
Authorization of 12 months may be granted for the treatment of essential thrombocythemia
in members who have had an inadequate response or loss of response to hydroxyurea,
peginterferon alfa-2a, or anagrelide.
J. Myeloid/Lymphoid Neoplasms with Eosinophilia
Authorization of 12 months may be granted for the treatment of myeloid and/or lymphoid
neoplasms with eosinophilia and JAK2 rearrangement in the chronic phase or blast phase.
K. Cytokine Release Syndrome
Authorization of 1 month may be granted for treatment of chimeric antigen receptor (CAR)
T-cell-induced cytokine release syndrome that is refractory to high-dose corticosteroids and
anti-IL-6 therapy.
L. Concomitant Myositis and Myocarditis
Authorization of 1 month may be granted for treatment of immune checkpoint inhibitor-
related concomitant myositis and myocarditis when requested agent is used in combination
with abatacept.
IV. CONTINUATION OF THERAPY
A. Myelofibrosis, Accelerated/Blast Phase Myeloproliferative Neoplasms, Polycythemia
Vera, Acute GVHD, Chronic GVHD, and Essential Thrombocythemia
Authorization of 12 months may be granted for continued treatment in members requesting
reauthorization who have improvement in symptoms and no unacceptable toxicity.
B. Acute Lymphoblastic Leukemia (ALL)/Lymphoblastic Lymphoma (LL), Atypical
Chronic Myeloid Leukemia (aCML) / Myelodysplastic/Myeloproliferative Neoplasms
(MDS/MPN) with Neutrophilia, Chronic Myelomonocytic Leukemia (CMML)-2, T-Cell
Prolymphocytic Leukemia, T-Cell Large Granular Lymphocytic Leukemia, and
Myeloid/Lymphoid Neoplasms with Eosinophilia
Authorization of 12 months may be granted for continued treatment in members requesting
reauthorization when there is no evidence of unacceptable toxicity or disease progression
while on the current regimen.
C. Cytokine Release Syndrome and Concomitant Myositis and Myocarditis
All members (including new members) requesting authorization for continuation of therapy
must meet all initial authorization criteria.
Place of Service:
Outpatient
The above policy is based on the following references:
- Jakafi [package insert]. Wilmington, DE: Incyte Corporation; January 2023.
- The NCCN Drugs & Biologics Compendium® © 2024 National Comprehensive Cancer Network, Inc. Available at: http://www.nccn.org. Accessed March 19, 2024.
- Zeiser R, Burchert A, Lengerke C, et al: Ruxolitinib in corticosteroid-refractory graft-versus-host disease after allogeneic stem cell transplantation: a multicenter survey. Leukemia 2015; 29(10):2062-2068.
- Zeiser R, Burchert A, Lengerke C, et al: Long-term follow-up of patients with corticosteroid-refractory graft-versus-host disease treated with ruxolitinib. Blood 2016; 128(22):4561
- Raetz Elizabeth, Loh Mignon. A Phase 2 Study of the JAK1/JAK2 Inhibitor Ruxolitinib with Chemotherapy in Children with De Novo High-Risk CRLF2-Rearranged and/or JAK Pathway-Mutant Acute Lymphoblastic Leukemia. American Society of Hematology. 2016: 13(3).
- Ding YY, Stern JW, Jubelirer TF, et al. Clinical efficacy of Ruxolitinib and chemotherapy in a child with Philadelphia chromosome-like acute lymphoblastic leukemia with GOLGAS-JAK2 fusion and induction failure. 2018 Sep;103(9):e427-e431. doi: 10.3324/haematol.2018.192088. Epub 2018 May 17.
- National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology: Pediatric Acute Lymphoblastic Leukemia. Available at: http://www.nccn.org. Version 3.2024. https://www.nccn.org/professionals/physician_gls/pdf/ped_all.pdf. Accessed January 10, 2024.
Copyright Aetna Inc. All rights reserved. Pharmacy Clinical Policy Bulletins are developed by Aetna to assist in administering plan benefits and constitute neither offers of coverage nor medical advice. This Clinical Policy Bulletin contains only a partial, general description of plan or program benefits and does not constitute a contract. Aetna does not provide health care services and, therefore, cannot guarantee any results or outcomes. Participating providers are independent contractors in private practice and are neither employees nor agents of Aetna or its affiliates. Treating providers are solely responsible for medical advice and treatment of members. This Clinical Policy Bulletin may be updated and therefore is subject to change.
July 20, 2024
