Primary Myelofibrosis

Primary Myelofibrosis (PMF) is a chronic myeloproliferative disorder characterized by bone marrow fibrosis, splenomegaly, and anemia with nucleated and teardrop-shaped RBCs. The spleen is often very large. Myelofibrosis may be primary or secondary to a number of hematologic, malignant, and nonmalignant conditions e.g. PRV, ET, hairy cell leukemia, cancer with bone marrow metastases, osteomyelitis and TB. PMF is more common than […]






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Essential Thrombocythemia

Essential thrombocythaemia (ET) is a myeloproliferative neoplasm (MPN) characterised by megakaryocyte hyperplasia and thrombocytosis due to a clonal abnormality of a multipotent hematopoietic stem cell. 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 is asymptomatic at diagnosis. ET usually occurs […]






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Polycythemia Rubra Vera

Polycythemia Rubra Vera (PRV) or Primary Polycythemia Vera is a chronic myeloproliferative disorder characterized by an increase in RBC mass, which often manifests as an increased hematocrit (Hct). There is a malignant transformation of the multipotential stem cell with excess production of red cells. PRV involves increased production of all cell lines, including RBCs, WBCs, and platelets. Polycythaemia Rubra Vera […]






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Chronic Myeloid Leukemia

Aetiology: In chronic myeloid leukemia (CML) there is a malignant transformation of the multipotential hematopoietic stem cells (pluripotent stem cells). There are excessive cells in the granulocytic series, primarily in the bone marrow but also in extramedullary sites (e.g. spleen, liver), although these malignant cells do differentiate nearly normally. Although granulocyte production predominates, the neoplastic clone includes RBCs, megakaryocytes, monocytes, and […]






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