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15 Cards in this Set
- Front
- Back
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etiol
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clonal disorder of hemopoietic stem cells assoc with deletetion of chromos 5q and 7>>>>ineffective erytropoesis
with crowded hypercellular marrow and pancytopenia |
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clinical
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usually > 5o years old of age with pancytopenia and < 20 % blasts>>>> respective signs and symptoms
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ds/ most specific finding
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peripheral smear may show macroovlocytes and / or bilobed neutrophill ( Pelger_ Huet anomaly)
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gold standard test
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BM biopsy
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can it progress to leukemia
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yes if > 20 % blasts
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management
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if P < 60 and has a matched donor>>> BMT - first line
other wise management is individualized but should include" erythropoetin and transfus as needed drug therapy with lenalidomide ( FOR MYELODISPL SYNDROME WITH 5q abnormality) immunomodulator/ antiangiogenic and azacitidine( toxic for renal system!, MOA-hypomethilaion of DNA and direc toxic effect on abnl hematopoetic cells in bone marrow) if indicated |
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def
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per smear and lab finding
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psudo peger huet anomaly
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dimorphic population of red cells( small and big)
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ds
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sideroblasts
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ds... more blasts- worse... could turn to acute leukemia
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5 q minus syndrome
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instead of having thrombocytopenia- have trhombocytosis
bone marrow- dysplastic this is genetic disease |
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tx
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best supportive care if anemia- give transf
if fever- antibiotics about tranplsntation- majority of patients are old-- wont work for them |
