![]() AlloHSCT for PMF is potentially curative, but still hazardous. ĭrug therapy for PMF is not effective, in terms of overall or event-free survival. The risk factors for leukemia-free survival include ≥3% circulating blasts, platelet count <100 x 109/L, and presence of unfavorable karyotype. Prognostic aspects are derived from a concept of PMF as a heterogeneous disorder with rather individual manifestation and evolution, with highly variable median survival of up to >20 years.Īn appropriate scoring scale (IPSS) uses five risk factors to predict prognosis and stratify the patients into risk groups. In addition to JAK2 V617F or MPL mutations, a more recently found calreticulin (CALR) gene marker have been proposed for the MF (and essential patients with MF and ET). Differential diagnosis of PMF should also consider marrow fibrosis caused by other non-neoplastic or neoplastic conditions. This disorder should be discerned from other myeloid neoplasias, i.e., polycythemia vera and essential thrombocythemia (ET). PMF diagnosis is performed by appropriate WHO criteria and includes clinical, morphologic, cytogenetic, and molecular parameters. Meanwhile, allogeneic hemopoietic stem cell transplantation (allo-HSCT) is the only curative procedure for the PMF. ![]() A conventional cytostatic therapy of PMF does not affect the survival rates. ![]() Acute leukemia develops in up to 30% of PMF. PMF may be accomplished by gradual bone marrow failure, splenomegaly, severe general (constitutional) signs, and clinical features of extramedullary hemopoiesis. Primary myelofibrosis (PMF) is BCR/ABL–negative myeloproliferative disorder with splenomegaly, leukoerythroblasts, extramedullary hematopoiesis, reactive bone marrow fibrosis and neoangiogenesis with abnormal cytokine production, generally, affecting elderly persons. ![]()
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