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Myelodysplastic Syndrome

Myelodysplastic Syndrome. Myelodysplastic Syndrome. Group of clonal disorders of hematopoietic stem cells characterized by cellular dysplasia and ineffective hematopoiesis Results in cytopenias and dysmyelopoiesis Variable risk of transformation to acute leukemia. Pathophysiology.

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Myelodysplastic Syndrome

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  1. Myelodysplastic Syndrome

  2. Myelodysplastic Syndrome • Group of clonal disorders of hematopoietic stem cells characterized by cellular dysplasia and ineffective hematopoiesis • Results in cytopenias and dysmyelopoiesis • Variable risk of transformation to acute leukemia

  3. Pathophysiology • Primary MDS or Secondary MDS • A clonal mutation in hematopoietic stem cells • Bone marrow failure due to ineffective hematopoiesis • Increased cytokines (TNF-a) inhibit hematopoiesis • Increased apoptosis • Results in futile cycling of blood cells with impaired production of mature blood cells

  4. Prevalence • Mainly a disorder of older patients, rarely presents before age 50 • Median age is 65 years • Male predominance

  5. Clinical Presentation Non specific signs and symptoms of various cytopenias • Fatigue, SOB, exercise intolerance, pallor, tachycardia • Mucosal bleeding, petechiae, ecchymoses • Infection, fever B-symptoms are uncommon Splenomegaly and lymphadenopathy are rare

  6. Diagnosis • Anemia uniformly present with decreased reticulocytosis • >50% present with pancytopenia • 50% present with associated neutropenia • <5% isolated neutropenia or thrombocytopenia • Cytopenia with normal or hypercellular bone marrow with single or multi-lineage dysplasia

  7. Morphology • Peripheral changes include oval macrocytic red cells, hypogranular granulocytes with the pseudo-Pelger–Huët anomaly, and giant platelets.

  8. Morphology • Megaloblastic red-cell precursors with multiple nuclei or asynchronous maturation of the nucleus and the cytoplasm

  9. Morphology • Ringed sideroblasts, erythroid precursors with iron-laden mitochondria

  10. Classification

  11. Classification Cont.

  12. Classification cont.

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