Subject: Jakafi 1999-A SGM P2022
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. Acute and Chronic GVHD
6. Chronic myelomonocytic leukemia (CMML)-2
7. BCR-ABL negative atypical chronic myeloid leukemia
(aCML)
8. Essential Thrombocythemia
9. Myeloid/lymphoid neoplasms with eosinophilia and JAK2
rearrangement in chronic phase
10. Myeloid, lymphoid, or mixed lineage neoplasms with
eosinophilia and JAK2 rearrangement in blast phase
11. 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)
Authorization of 12 months may be granted for the
treatment of BCR-ABL negative atypical chronic myeloid
leukemia (aCML) 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), 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; September 2021.
- The NCCN Drugs & Biologics Compendium® © 2022 National Comprehensive Cancer Network, Inc. Available at: http://www.nccn.org. Accessed January 7, 2022.
- 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 1.2022. https://www.nccn.org/professionals/physician_gls/pdf/ped_all.pdf. Accessed July 8, 2020.
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 26, 2022
