TY - JOUR
T1 - Managing myelofibrosis (MF) that "blasts" through
T2 - Advancements in the treatment of relapsed/refractory and blast-phase MF
AU - Scherber, Robyn M.
AU - Mesa, Ruben A.
N1 - Publisher Copyright:
© 2018 American Society of Hematology. All rights reserved.
PY - 2018/11/30
Y1 - 2018/11/30
N2 - Myelofibrosis (MF) is themost aggressive form of Philadelphia chromosome-negative myeloproliferative neoplasm, and it is complicated by severe symptom burden, thrombotic events, infections, cytopenias, and transformation to acute myeloid leukemia (AML). Ruxolitinib, the first-line therapy for symptomatic or intermediate- and high-prognostic risk MF, has improved overall survival for this population. However, approximately one-half of MF patients will discontinue ruxolitinib by the first few years of therapy due to a spectrum of resistance, intolerance, relapse, or progression to blast phase disease. Danazol, erythropoietin-stimulating agents, and spleen-directed therapies can be useful in the ruxolitinib-resistant setting. In the ruxolitinib-refractory or -intolerant setting, commercial and novel therapies, either alone or in combination with ruxolitinib, have shown clinical utility. For blast-phase MF, the recent advancements in available AML therapies have increased the options with targeted and more tolerable therapies. In this article, we will discuss our paradigm for the management of relapsed/ refractory and blast-phase MF in the context of therapeutic advancements in both AML and MF.
AB - Myelofibrosis (MF) is themost aggressive form of Philadelphia chromosome-negative myeloproliferative neoplasm, and it is complicated by severe symptom burden, thrombotic events, infections, cytopenias, and transformation to acute myeloid leukemia (AML). Ruxolitinib, the first-line therapy for symptomatic or intermediate- and high-prognostic risk MF, has improved overall survival for this population. However, approximately one-half of MF patients will discontinue ruxolitinib by the first few years of therapy due to a spectrum of resistance, intolerance, relapse, or progression to blast phase disease. Danazol, erythropoietin-stimulating agents, and spleen-directed therapies can be useful in the ruxolitinib-resistant setting. In the ruxolitinib-refractory or -intolerant setting, commercial and novel therapies, either alone or in combination with ruxolitinib, have shown clinical utility. For blast-phase MF, the recent advancements in available AML therapies have increased the options with targeted and more tolerable therapies. In this article, we will discuss our paradigm for the management of relapsed/ refractory and blast-phase MF in the context of therapeutic advancements in both AML and MF.
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U2 - 10.1182/asheducation-2018.1.118
DO - 10.1182/asheducation-2018.1.118
M3 - Article
C2 - 30504300
AN - SCOPUS:85058735177
SN - 1520-4391
VL - 2018
SP - 118
EP - 126
JO - Hematology (United States)
JF - Hematology (United States)
IS - 1
ER -