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17 Cards in this Set
- Front
- Back
heteogenous group of clonal stem cell disorders.
ineffective hamatopoiesis. can>AML mostly older pts, >60 |
Myelodysplastic syndrome
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aka "anemia of the elderly"
seen with lymphadenopathy or splenomegaly |
Myelodysplastic syndrome
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have unexplained cytopenias and dysplasia among 2 or more cell lines
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Myelodysplastic syndrome
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Myelodysplastic syndrome:
blood smear |
macrocytic, hypogranular neutrophils
Pelger Huet nuclei circulating micromegakaryocytes large, granular lymphs |
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Myelodysplastic syndrome:
bone marrow |
hypercellular (80%) or hypocellular (20%)
abn. immature blasts increased myeloblasts and dysplasia in megakaryocytes and erythroid cells ringed sideroblasts |
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IPSS
scoring for blasts |
<5 = 0
5-10 = 0.5 11-20 = 1.5 |
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IPSS
scoring for cytopenia |
0-1 = 0
2-3 = 0.5 |
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WHO Class.:
RA |
% cases- 5-10
Periph blood- Anemia, <1% blasts, decreased monos Bone marrow- Erythroid dysplasia only, <5% blast < 15% ringed sideroblasts Rate of AML prog- 6% |
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WHO class:
RARS |
% cases- 10-15
Periph blood- Anemia, <1% blasts, decreased monos Bone marrow- Erythoid dysplasia with greater than 15% ringed sideroblasts Rate of AML prog- 1-2% |
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WHO class:
RCMD |
% cases- 11
Periph blood- Bicytopenia or pancytopenia Rare blasts and decreased monocytes Bone marrow- Dysplasia in > 10% of cells in 2 cell lines, <5% blasts in marrow, no Auer Rods, <15% ringed sideroblasts Rate of AML prog- 11% |
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WHO class:
RCMD-RS |
% cases- 15
Peripheral blood-Bicytopenia or pancytopenia Rare blasts and decreased monocytes Bone marrow-Dysplasia in >10% of cells in 2 cell lines, >15% ringed sideroblasts, <5% myeloblasts Rate of AML prog- 11% |
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WHO class:
RAEB 1 |
% cases- 20
Peripheral blood-Cytopenias, decreased monocytes, <5% blasts no Auer Rods Bone marrow- Multilineage dysplasias 5-9% blasts with no Auer Rods Rate of AML prog- 25% |
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WHO class:
RAEB 2 |
% cases- 20
Peripheral blood- Cytopenias 5-19% blasts with Auer Rods Bone marrow- 10-19% Blasts with +/- Auer Rods Rate of AML prog- 33% |
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WHO class:
MDS-U |
Peripheral blood- Cytopenia, rare blasts
Bone marrow- Unilineage dysplasias in granulocytes or megakaryocytes, No Auer Rods |
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WHO class:
5q Syn |
Peripheral blood- Anemia, <5%blasts normal to increased platelets
Bone marrow- Normal to increased megakaryocytes <5% blasts No Auer Rods Isolated del(5q) |
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Myelodysplastic syndorme:
diagnosis |
hypocellular marrow and dysplastic marrow
CMML |
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MDS:
Therapy |
transfusion, anitmicrobials, iron chelation, C-CSF, GM-CSF, growth factors
suppresion of MDS clone and increase bm function some stem cell transplant |