Category: Myeloproliferative Disorders

Primary Myelofibrosis (PMF) is a chronic myeloproliferative neoplasm characterized by bone marrow fibrosis, splenomegaly, and anemia with nucleated and teardrop-shaped RBCs. The spleen is often very large. Marked enlargement of the spleen and liver may result in infarction, portal hypertension, hypersplenism, plasma volume expansion, and splanchnic vein thrombosis. Myelofibrosis may be primary or secondary to several hematologic, malignant, and non-malignant […]

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Essential thrombocythemia (ET) is a myeloproliferative neoplasm (MPN) characterized by megakaryocyte hyperplasia and thrombocytosis due to a multipotent hematopoietic stem cell clonal abnormality. From the genetic perspective, ET patients harbour mutations in JAK2 (50–60%), CALR (15–30%) and MPL (1–5%) genes. Platelet survival is normal in ET. Approximately one-third of patients with ET are asymptomatic at diagnosis. ET usually occurs with bimodal […]

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Polycythemia Rubra Vera (PRV) is a chronic myeloproliferative neoplasm characterized by an increased red blood cell (RBC) production and RBC mass often manifested as an increased hematocrit (Hct).  There is a malignant transformation of the multipotential stem cell with excess production of red blood cells in the bone marrow and blood, causing the blood to thicken. The number of white […]

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