myelodysplastic synd_06-07.ppt
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MYELODYSPLASTIC SYNDROMEMYELODYSPLASTIC SYNDROME
Masatoshi Kida, M.D.Dept. of Pathology
University of Vermont
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Myelodysplastic SyndromeMyelodysplastic 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)
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Myelodysplastic SyndromeMyelodysplastic Syndrome2001 WHO classification2001 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
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myelodysplastic syndromemyelodysplastic syndromeclassificationclassification
RA <5% <1% --
RARS <5% <1% +
RAEB 5-20% <5%
RAEB-T 20-30% >5%
CMML dysplastic monocytosis
blasts ringed sideroblasts
BM peripheral
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myelodysplastic syndromemyelodysplastic syndrome
Etiology:• vast majority of cases are sporadic
“risk factors”• chemical solvent (benzene)• pesticides• chemotherapy / radiation therapy
“therapy-related MDS/AML”
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myelodysplastic syndromemyelodysplastic 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
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myelodysplastic syndromemyelodysplastic syndromegenetic abnormalitiesgenetic 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
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myelodysplastic syndromemyelodysplastic syndromeclinical featuresclinical 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
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Myelodysplastic SyndromeMyelodysplastic Syndrome
•anemia•macrocytosis•large plts without granules•PMNs
•hypogranulated•pseudo Pelger-Huët cells•abnormally segmented nuclei•Döhl bodies
•circulating myeloblasts
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myelodysplastic syndromemyelodysplastic syndromeclinical featuresclinical 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
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myelodysplastic syndromemyelodysplastic syndrometreatmenttreatment
1. supportive care (blood transfusion, etc.)
2. allogeneic bone marrow transplant
42% disease-free survival at 4 years
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