myelodysplastic synd_06-07.ppt
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myelodysplastic synd_06-07.ppt Presentation Transcript

  • 1. MYELODYSPLASTIC SYNDROME Masatoshi Kida, M.D. Dept. of Pathology University of Vermont
  • 2. Myelodysplastic Syndrome
    • heterogeneous group of hematologic disorders
    • maturation defects resulting in ineffective hematopoiesis  pancytopenia in peripheral blood
    • “ preleukemia”  many progress to overt acute leukemia
    • classification (FAB classification)
    • (based on % blasts and ringed sideroblasts)
    • 1. refractory anemia (RA)
    • 2. refractory anemia with ringed sideroblasts (RARS)
    • 3. refractory anemia with excess blasts (RAEB)
    • 4. refractory anemia with excess blasts in transformation (RAEB-T)
    • 5. CMML (chronic myelomonocytic leukemia)
  • 3. Myelodysplastic Syndrome 2001 WHO classification
    • refractory anemia (RA)
    • refractory anemia with ringed sideroblasts (RARS)
    • refractory cytopenia with multilineage dysplasia (RCMD)
    • RCMD with ringed sideroblasts (RCMD-RS)
    • refractory anemia with excess blasts-1 (RAEB-1) <5% blasts
    • refractory anemia with excess blasts-2 (RAEB-2) 5-19% blasts
    • myelodysplastic syndrome, unclassified (MDS-U)
    • MDS with isolated del(5q) <5% blasts
  • 4. myelodysplastic syndrome classification
    • RA <5% <1% --
    • RARS <5% <1% +
    • RAEB 5-20% <5%
    • RAEB-T 20-30% >5%
    • CMML dysplastic monocytosis
    blasts ringed sideroblasts BM peripheral
  • 5. myelodysplastic syndrome
    • Etiology:
      • vast majority of cases are sporadic
    • “risk factors”
      • chemical solvent (benzene)
      • pesticides
      • chemotherapy / radiation therapy
    • “therapy-related MDS/AML”
  • 6. myelodysplastic syndrome
    • Incidence:
      • de novo MDS 1 per 100,000 per year
      • (>60 y/o --- 25 to 50 per 100,000 per year)
      • t-MDS/AML 4 to 18% in autologous BMT
  • 7. myelodysplastic syndrome genetic abnormalities
    • “ loss of genetic material”
      • 5q- 15% of de novo MDS 50% of t-MDS
      • monosomy 7
      • trisomy 8
      • 21q-
      • 17q-
      • 20q-
      • t(11;16)(q23;p13) -- exclusively with t-MDS/AML
      • t(3;21)(q22;q22) -- with some of t-MDS/AML
      • t(5;12)(q33;p13) -- CMML
  • 8. myelodysplastic syndrome clinical features
    • peripheral cytopenia
    • neutropenia 24 to 39% of patients
    • anemia 45 to 93%
    • thrombocytopenia 28 to 45%
    • both quantitative and qualitative defect
    • highly susceptible to bacterial infection
    • normal T- and B-cell numbers and functions
    • no opportunistic infection
  • 9. Myelodysplastic Syndrome
    • anemia
    • macrocytosis
    • large plts without granules
    • PMNs
      • hypogranulated
      • pseudo Pelger-Huët cells
      • abnormally segmented nuclei
      • Döhl bodies
    • circulating myeloblasts
  • 10. myelodysplastic syndrome clinical features
    • de novo MDS -- occurs in elderly population
    • 30% die of unrelated underlying disease(s)
    • 40% die of complication(s) of marrow failure
    • 30% die of transformation to acute leukemia
  • 11. myelodysplastic syndrome treatment
    • supportive care (blood transfusion, etc.)
    • allogeneic bone marrow transplant
    • 42% disease-free survival at 4 years
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