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MYELODYSPLASTIC SYNDROME www.freelivedoctor.com
Myelodysplastic Syndrome <ul><li>heterogeneous group of hematologic disorders </li></ul><ul><li>maturation defects resulti...
Myelodysplastic Syndrome 2001 WHO classification <ul><li>refractory anemia (RA) </li></ul><ul><li>refractory anemia with r...
myelodysplastic syndrome classification <ul><li>RA <5% <1% -- </li></ul><ul><li>RARS <5% <1% + </li></ul><ul><li>RAEB 5-20...
myelodysplastic syndrome <ul><li>Etiology: </li></ul><ul><ul><li>vast majority of cases are sporadic </li></ul></ul><ul><l...
myelodysplastic syndrome <ul><li>Incidence: </li></ul><ul><ul><li>de novo MDS 1 per 100,000 per year </li></ul></ul><ul><u...
myelodysplastic syndrome genetic abnormalities <ul><li>“ loss of genetic material” </li></ul><ul><ul><li>5q- 15% of de nov...
myelodysplastic syndrome clinical features <ul><li>peripheral cytopenia </li></ul><ul><li>neutropenia 24 to 39% of patient...
Myelodysplastic Syndrome <ul><li>anemia </li></ul><ul><li>macrocytosis </li></ul><ul><li>large plts without granules </li>...
myelodysplastic syndrome clinical features <ul><li>de novo MDS -- occurs in elderly population </li></ul><ul><li>30% die o...
myelodysplastic syndrome treatment <ul><li>supportive care (blood transfusion, etc.) </li></ul><ul><li>allogeneic  bone ma...
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Myelodysplastic syndrome

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Transcript of "Myelodysplastic syndrome"

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