Diamond-Blackfan syndrome: evidence against cell-mediated erythropoietic suppression.

Lymphocyte dysfunction in congenital hypoplastic anemia.

Congenital hypoplastic anemia (Diamond-Blackfan syndrome) is thought to involve the erythropoietic cell line alone. In this study, the evaluation of lymphocyte function in five patients with this syndrome revealed a number of abnormalities. Peripheral blood T lymphocyte percentages as assessed by monoclonal antibodies were decreased in three patients. T-helper/T-suppressor cell (OKT4:OKT8) rati...

Effect of Diamond - Blackfan Syndrome Peripheral Blood Lymphocytes on CFU - E Numbers

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 c...

Saunders erythropoietic suppression Diamond - Blackfan syndrome

http://bloodjournal.hematologylibrary.org/site/misc/rights.xhtml#repub_requests Information about reproducing this article in parts or in its entirety may be found online at: http://bloodjournal.hematologylibrary.org/site/misc/rights.xhtml#reprints Information about ordering reprints may be found online at: http://bloodjournal.hematologylibrary.org/site/subscriptions/index.xhtml Information abo...

Functional Links Between Glucocorticoids and Cytokines In DBA

Diamond Blackfan anaemia (DBA) is a red blood cell aplasia characterized by erythropoietic defects as well as congenital anomalies. Forty percent of patients with DBA are treated with glucocorticoid steroids, which remain the mainstay of treatment in DBA. Many advances in the understanding of the physiological role of the glucocorticoid receptor have been made since the first introduction of gl...

Hepcidin levels in Diamond-Blackfan anemia reflect erythropoietic activity and transfusion dependency.