Abstract
Cytopenias represent a frequently encountered and often difficult clinical problem in patients with myelofibrosis (MF), and are usually more pronounced in patients with primary myelofibrosis (PMF) than those with post-polycythemia vera (post-PV) or post-essential thrombocythemia (post-ET) MF. Anemia is present in approximately 30% of patients with PMF at diagnosis and eventually develops in essentially all patients. While severe thrombocytopenia (platelets <50 x 109/L) in patients with MF is uncommon, it is a well-recognized poor prognostic factor. Leukopenia is less frequent of a problem in the clinic, and may associate with specific cytogenetic abnormalities, e.g., 20q-, +8 and -7/7q-. Importantly, on-target anemia and thrombocytopenia are frequently observed during therapy with the JAK1/2 inhibitor ruxolitinib, especially during the first 12-24 weeks on therapy. Careful dose adjustment of ruxolitinib without premature discontinuation is critical while managing these cytopenias, as spleen responses to ruxolitinib are dose-dependent and correlate with survival in patients with advanced MF. Splenic irradiation for recalcitrant splenomegaly, although only infrequently performed in the ruxolitinib era, can result in persistent and profound cytopenias.
Published Version
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