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Myeloproliferative Disorders

Myeloproliferative disorders are crucial topics for FMGE, NEET PG, and USMLE Step 1 exams, illuminating the realm of hematologic pathology. These disorders encompass a group of clonal hematopoietic stem cell disorders characterized by excessive proliferation of one or more myeloid cell lineages, leading to increased production of mature blood cells. ⚠ Common myeloproliferative disorders include polycythemia vera (PV), essential thrombocythemia (ET), primary myelofibrosis (PMF), and chronic myeloid leukemia (CML). PV is characterized by an increase in red blood cell mass, whereas ET involves elevated platelet counts. PMF is characterized by bone marrow fibrosis, leading to cytopenias and splenomegaly, while CML is associated with the presence of the Philadelphia chromosome (Ph chromosome) and the BCRABL fusion gene, resulting in uncontrolled granulocytic proliferation. Diagnosis relies on peripheral blood smear examination, bone marrow biopsy, and genetic testing to determine specific molecular abnormalities. Treatment modalities encompass targeted therapy, cytoreductive agents, and stem cell transplantation, tailored to the individual patient's risk stratification and disease characteristics. Understanding the pathogenesis, clinical manifestations, and therapeutic approaches to myeloproliferative disorders is essential for medical professionals navigating the landscape of hematologic malignancies. Let's delve into these disorders and unlock the mysteries of hematopoiesis gone awry!

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