Myelofibrosis with myeloid metaplasia (MMM), Idiopathic myelofibrosis, Agnogenic myeloid metaplasia
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چکیده
MMM usually presents with fatigue, weight loss, splenomegaly with or without symptoms. Anemia and various alterations of the white blood cell and/or platelet count are frequently seen at diagnosis. Thrombocytopenia-related bleeding may occur. MMM must be distinguished from myelodysplasia with fibrosis, from acute megakayoblastic leukemia and acute myelofibrosis. As the disease progresses, increased marrow fibrosis with severe symptomatic peripheral cytopenias and extramedullary hemopoiesis predominate, with consequent massive splenomegaly, hepatomegaly with portal hypertension, pulmonary hypertension. Leukemic transformation may represent the terminal event in 5-20% of the cases.
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