Marrow Failure Syndromes: Marrow Failure Syndromes -Bone marrow failure indicates the inability of hematopoiesis to meet the demands of production.
-May be either congenital or acquired. www.HemOnc101.com 2
Diamond-Blackfan Anemia (DBA): Diamond-Blackfan Anemia (DBA) A 2 month old infant presenting with craniofacial malformations, thumb as well as upper limb abnormalities, cardiac defects, urogenital malformations, and a cleft palate. WBC and platelet count normal but hemoglobin 5g/dL, macrocytic with low reticulocyte count. Negative Coombs’ test, normal haptolgobin, normal B12, folate, TSH. Bone marrow reveals normal myeloid cells and megakaryocytes but marked reduction in immature proerythroblasts.
-DBA is a congenital erythroid aplasia which usually presents in infancy. www.HemOnc101.com 3