Subject: Jakafi 1999-A SGM P2023
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. Accelerated phase or blast phase myelofibrosis/acute
myeloid leukemia
3. Polycythemia vera in patients with inadequate response
or loss of response to interferon therapy
4. Philadelphia chromosome (Ph-like) B-cell Acute
Lymphoblastic Leukemia (ALL)/Lymphoblastic lymphoma
(LL)
5. Chronic myelomonocytic leukemia (CMML)-2
6. BCR-ABL negative atypical chronic myeloid leukemia
(aCML) / Myelodysplastic/Myeloproliferative Neoplasms
(MDS/MPN) with neutrophilia
7. Essential Thrombocythemia
8. Myeloid/lymphoid neoplasms with eosinophilia and JAK2
rearrangement in blast phase or chronic phase
9. CAR T-cell-related toxicities - Cytokine release syndrome
(CRS)
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/Acute Myeloid Leukemia
Authorization of 12 months may be granted for the treatment
of myelofibrosis/acute myeloid leukemia.
B. Polycythemia Vera
Authorization of 12 months may be granted for the treatment
of polycythemia vera in members who have had an
inadequate response or intolerance to hydroxyurea or
peginterferon alfa-2a.
C. 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
D. 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.
E. 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.
F. 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.
G. Essential Thrombocythemia
Authorization of 12 months may be granted for the treatment
of essential thrombocythemia in members who have had an
inadequate response or intolerance to hydroxyurea,
peginterferon alfa-2a, or anagrelide.
H. 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.
I. 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.
IV. CONTINUATION OF THERAPY
A. Myelofibrosis/Acute Myeloid Leukemia, 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, 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
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; December 2022.
- The NCCN Drugs & Biologics Compendium® © 2023 National Comprehensive Cancer Network, Inc. Available at: http://www.nccn.org. Accessed January 10, 2023.
- 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 1.2023. https://www.nccn.org/professionals/physician_gls/pdf/ped_all.pdf. Accessed January 10, 2023.
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.
June 18, 2023
