Familial myelofibrosis.

Myelofibrosis with myeloid metaplasia (MMM), Idiopathic myelofibrosis, Agnogenic myeloid metaplasia

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, incre...

[Myelofibrosis with myeloid metaplasia].

Introduction Myelofibrosis with myeloid metaplasia (MMM) represents both agnogenic myeloid metaplasia (AMM) and the fibrotic stages of polycythemia vera (PV) and essential thrombocythemia (ET). The latter two conditions are also referred to as post polycythemic myelofibrosis (PPM) and post thrombocythemic myelofibrosis (PTM). MMM is currently classified under the broad category of chronic myelo...

Modern management of myelofibrosis.

The conventional treatment of myelofibrosis involves a wait-and-see approach for asymptomatic patients, oral chemotherapy for the hyperproliferative forms of the disease, androgens or erythropoietin for the anaemia, and splenectomy in selected patients. Low-dose thalidomide plus prednisone is a well-tolerated therapy for the anaemia and the thrombocytopenia of myelofibrosis, whereas imatinib ha...

Thalidomid in Idiopathic Myelofibrosis

How I treat myelofibrosis.

It is currently assumed that myelofibrosis (MF) originates from acquired mutations that target the hematopoietic stem cell and induce dysregulation of kinase signaling, clonal myeloproliferation, and abnormal cytokine expression. These pathogenetic processes are interdependent and also individually contributory to disease phenotype-bone marrow stromal changes, extramedullary hematopoiesis, inef...