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- W1973727425 endingPage "1077" @default.
- W1973727425 startingPage "1061" @default.
- W1973727425 abstract "Diamond-Blackfan anemia (DBA) is a rare congenital hypoplastic anemia32, 66 that usually presents early in infancy. Retrospective studies in the United Kingdom and the Netherlands are consistent with an incidence of 4–5 per million live births,12, 24 with evidence of inheritance (dominant and recessive) in perhaps 12% to 25% of cases.3, 48, 126 Congenital anomalies, in particular of the head and upper limbs, are present in about a quarter of reported patients. The disease is characterized by a moderate-to-severe macrocytic anemia, occasional neutropenia and/or thrombocytosis, and a normocellular bone marrow with erythroid hypoplasia. Although proerythroblasts may be present or even increased, normoblasts are usually markedly diminished or absent. The majority of patients (75%) respond to prednisone, and erythropoiesis can then be maintained in some cases with almost homeopathic doses of the drug. Nonresponders are usually transfusion-dependent, although responses to androgen have been observed.33, 58, 73, 110" @default.
- W1973727425 created "2016-06-24" @default.
- W1973727425 creator A5061126953 @default.
- W1973727425 creator A5062784258 @default.
- W1973727425 date "1997-12-01" @default.
- W1973727425 modified "2023-10-17" @default.
- W1973727425 title "DIAMOND-BLACKFAN ANEMIA" @default.
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