SynthesisAnnals of hematology2026
A systematic review of 47 published cases of extramedullary hematopoiesis in myelofibrosis: clinical patterns, treatment responses, and prognostic implications.
Synthesis in Annals of hematology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Extramedullary hematopoiesis (EMH) in myelofibrosis manifests beyond classical splenomegaly, causing life-threatening complications, yet lacks systematic characterization in the JAK inhibitor era. We systematically reviewed 36 publications (2011-2025), available on PubMed database, reporting 47 unique myelofibrosis-associated EMH cases, analyzing demographics, anatomic distribution, presentations, diagnostics, treatments, and outcomes.Median age was 68 years (range 43-82; M: F 26:21); primary myelofibrosis predominated (64%). EMH favored atypical sites: abdominopelvic (28%), thoracic (26%; pulmonary/pleural), serosal (17%), cutaneous (13%). Symptomatic onset occurred in 75% (dyspnea 28%, abdominal pain 21%). CT/MRI revealed characteristic lobulated masses with T2 hypointensity; biopsy confirmed trilineage hematopoiesis with atypical megakaryocytes (CD61+). Ruxolitinib-based regimens, administered in 19 patients (40%) as EMH-directed therapy, yielded rapid palliation (dyspnea/ascites resolution), complemented by radiotherapy (11%) and splenectomy; HSCT achieved complete remission in one case. Mortality reached 38% (median follow-up 5 months), driven by EMH-related organ failure or AML transformation. Atypical EMH signals advanced myelofibrosis with dismal prognosis, responsive to JAK inhibitors/locoregional therapies but highlighting sanctuary biology. Systematic surveillance and transplant referral are imperative; prospective registries are needed to refine risk models and test novel antifibrotics.
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