Diamond-Blackfan syndrome: evidence against cell-mediated erythropoietic suppression
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چکیده
منابع مشابه
Diamond-Blackfan syndrome: evidence against cell-mediated erythropoietic suppression.
The profound anemia of Diamond-Blackfan syndrome (DBS) is due to marrow red cell failure, but the pathogenesis is not understood. Studies by others indicated cell-mediated erythropoietic suppression in this condition. To explore this mechanism further, Ficoll-Hypaque--separated peripheral blood lymphocytes (PBL) from four anemic untreated patients with DBS, or from normals were cocultured with ...
متن کاملSaunders erythropoietic suppression Diamond - Blackfan syndrome
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Neutropenia is a rare complication of Diamond-Blackfan syndrome (congenital hypoplastic anaemia). Three patients are reported: all had neutropenia as well as anaemia, and to investigate the cause of the neutropenia culture of bone marrow for granulocyte-macrophage colony forming cells (GMCFCs) was performed. Two cases had a low incidence of GMCFCs, but the third case had a high incidence. These...
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Mutations affecting genes encoding ribosomal proteins cause Diamond Blackfan anemia (DBA), a rare congenital syndrome associated with physical anomalies, short stature, red cell aplasia, and an increased risk of malignancy. p53 activation has been identified as a key component in the pathophysiology of DBA after cellular and molecular studies of knockdown cellular and animal models of DBA and o...
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ژورنال
عنوان ژورنال: Blood
سال: 1978
ISSN: 0006-4971,1528-0020
DOI: 10.1182/blood.v51.6.1125.1125