alexa Case Series- Heterogeneity of Primary Myelofibrosis

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Case Series- Heterogeneity of Primary Myelofibrosis

Primary myelofibrosis (PMF) is a myeloproliferative neoplasm characterized by stem cell derived clonal myeloproliferation and extramedullary haematopoiesis (EMH). Myelofibrosis can present as a de novo disorder or evolve secondary to previous polycythemia vera or essential thrombocythemia (post PVMF or post ETMF) [1]. Primary myelofibrosis (PMF) is the least frequent among the chronic myeloproliferative diseases.One study reported an estimated incidence of 1.5 per 100,000 per year 2 PMF occurs mainly in the middle aged and elderly population. The median age at presentation is 67 years [2]. The primary pathogenic mechanism is a clonal stem-cell disorder that leads to ineffective erythropoiesis, dysplastic-megakaryocytic hyperplasia, and an increase in neutrophils with predominance in immature forms [3,4]. The disease is clinically characterized by progressive anemia, constitutional symptoms and splenomegaly. The laboratory findings show a leuko-erythroblastic blood film, tear drop poikilocytosis, reticulin fibrosis in bone marrow and elevated levels of various inflammatory and pro-angiogenic cytokines [1] more


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