Myelodysplastic Syndromes Flashcards
Group of acquired clonal hematologic disorder that affect maturation of erythroid, myeloid, and or megakaryocytic lines. PROGRESSIVE PB CYTOPENIAS (MAY EVENTUALLY PROGRESS TO AML)
Myelodysplastic Syndromes
Mechanism of Myelodysplastic Syndromes
Hypomethylation of DNA followed by hypermethylation of DNA
MDS peripheral blood findings
- cytopenias
- anemia (normochromic normocytic or macrocytic anemia)
- dysplasia
MDS bone marrow findings
- Hypercellular
- Dysplasia
- ineffective hematopoiesis
- M:E ratio decreased
Is flow cytometry and cytogenetics diagnostic for MDS?
NO! important for providing prognostic risks and therapy but not diagnostic
Criteria for calling a ringed sideroblasts in the erythrocyte line
at least 5 iron granules that circle at least 1/3 of the nucleus
What are the most common lab findings in MDS?
macrocytic or normocytic anemia (with low retics)
MDS may present with _____ _____ RBCs but still have normal iron stores
microcytic hypochromic
MDS may present with megaloblastoid cellular development but still have _______ vit B12and folate values
normal
Most common myelodysplastic Syndromes (3)
- Refractory Anemia with Excess Blasts (RAEB-1 and 2)
- Refractory Cytopenia with Multilineage Dysplasia
- Refractory Cytopenia with Unilineage Dysplasia
PB Blasts in RAEB-1
PB Blasts in RAEB-2
5-19%
BM Blasts in RAEB-1
5-9%
BM Blasts in RAEB-2
10-19%
Myelodysplastic Syndrome with Isolated Del(5q)
- what deletion
- predominantly in what sex?
- what anemia is characteristic
- deletion of a 5q presumed tumor suppressor gene
- women
- macrocytic