Lymphocyte dysfunction in congenital hypoplastic anemia.

Congenital hypoplastic anemia (Diamond-Blackfan syndrome) terminating in acute myelogenous leukemia.

A 31-yr-old female with congenital hypoplastic anemia (Diamond-Blackfan syndrome) whose long course terminated in acute myelogenous leukemia is described. In contrast to Fanconi anemia, malignant transformation rarely occurs in congenital hypoplastic anemia. This patient's diagnosis of congenital hypoplastic anemia is supported by her clinical course, absence of renal abnormalities, a negative ...

Congenital hypoplastic anemia associated with multiple developmental defects (Fanconi syndrome).

Oral manifestations in congenital hypoplastic anemia (Diamond-Blackfan anemia): clinical report.

Congenital hypoplastic anemia is a rare disease characterized by a normochromic, normocytic anemia, a deficiency of erythroblasts in the marrow, and normal leukocyte and platelet counts. The disease usually is treated successfully with corticosteroid therapy; however, some patients become refractory to corticosteroids and must receive washed, packed red blood cell transfusions. The management a...

Congenital hypoplastic anemia inhibition of erythropoiesis by sera from patients with congenital hypoplastic anemia.

An in vitro marrow culture assay designed to measure erythropoietic capability was used to ascertain the presence of an inhibitor in the sera of patients with congenital hypoplastic anemia (CHA). Marrow cells from nine anemic CHA patients responded to the stimulatory effect of exogenous erythropoietin (EPO) by an increase in heme synthesis in the presence of normal serum. The effect on heme syn...

Prolonged response to cyclosporin-A in hypoplastic refractory anemia and correlation with in vitro studies.

BACKGROUND AND OBJECTIVE Lymphocyte abnormalities in myelodysplastic syndromes (MDS) have been widely described, but the role of the immune system in the pathogenesis of these clonal disorders remains controversial. An active role of lymphocytes in suppressing normal hematopoiesis may be implicated in MDS with hypoplastic marrow. We have studied in vitro and in vivo activity of cyclosporin-A (C...