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Week 12: Myelodysplastic Syndrome

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Presentation on theme: "Week 12: Myelodysplastic Syndrome"— Presentation transcript:

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 % 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 WHO Classification of MDS (1997)
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

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

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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