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Week 12: Myelodysplastic Syndrome

Week 12: Myelodysplastic Syndrome. Myelodysplasia Diserythropoiesis Dismyelopoiesis Dismegakaryopoiesis FAB calssification Ring Sideroblast Type I and II blasts WHO classification. Myelodysplastic Syndrome. Primary, neoplastic, pluripotential stem cell disorder

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Week 12: Myelodysplastic Syndrome

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  1. Week 12: Myelodysplastic Syndrome • Myelodysplasia • Diserythropoiesis • Dismyelopoiesis • Dismegakaryopoiesis • FAB calssification • Ring Sideroblast • Type I and II blasts • WHO classification

  2. Myelodysplastic Syndrome • Primary, neoplastic, pluripotential stem cell disorder • Severe cytopenias and maturation abnormality (dysplasia) in one or more cell lines • 30 - 40% of RAEB develop AML, mostly elderly • Evidence of impaired colony formation

  3. FAB Classification of MDS (1982) • Refractroy Anemia (RA) • RA with Ringed Sideroblasts (RARS) • RA with Excess Blasts (RAEB) • Chronic Meylomonocytic Leukemia (CMML) • RAEB in Transformation (RAEB-t)

  4. RA becomes RA Refractory cytopenia w/multilineage dysplasia (RCMD) -- complex karyotypes (+8, -7, 7q-, -5, 5q-, 20q-) RARS becomes RARS RCMD w/ ringed sideroblasts RAEB becomes RAEB types I and II 5q- Syndrome -- middle age to elderly females; deletion between bands 31 and 33; macrocytosis; good prognosis MDS, unclassifiable WHO Classification of MDS (1997)

  5. WHO Classification of MDS/MPS • Chronic Myelomonocytic Leukemia (CMML) -- from old FAB MDS • Atypical CML (aCML) -- no Ph chromosome • Juvenile Myelo/Myelomono Leukemia (JMML) • Note: FAB RAEB-t is reclassified as “AML w/multilineage dysplasia following MDS” because definition of AML is 20% blasts with WHO

  6. Dyserythropoiesis • Anemia • Normocytic or macro-ovalocytes • Low retic count • NRBC • Megaloblastic changes • Ringed sideroblast • Pappenheimer bodies • Basophilic stippling

  7. Dyserythropoiesis Ringed sideroblast

  8. Dysmyelopoiesis • Neutropenia • Monocytosis • Pseudo Pelger-Huet • Hypogranular PMN • <20% blasts in BM • Type I and type II blasts

  9. Dysgranulopoiesis

  10. Type I blasts No granules Prominent nucleoli Central nucleus Type II and III blasts Few 1o granules Prominent nucleoli Central nucleus Auer rods Promyelocyte Many 1o granules Less prominent nucleoli Eccentric nucleus Blasts in MDS

  11. Type I Blast Type III Blast

  12. CMML and NSE

  13. Dysmegakaryopoiesis • Low platelet count • Giant Platelet • Dwarf (micro) megakaryocyte

  14. Micromegakaryocyte Abnormal platelets

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