Natural history of idiopathic refractory sideroblastic anemia

Natural history of idiopathic refractory sideroblastic anemia.

We analyzed the natural history of idiopathic refractory sideroblastic anemia (IRSA) in 37 patients studied between 1969 and 1986. Although erythroid abnormalities were prominent in all, 12 patients also showed involvement of the granulocytic and/or megakaryocytic cell lines, and nonrandom chromosomal aberrations were observed in five of 23 patients studied for such defects. Measurements of ery...

Association of HLA-linked hemochromatosis with idiopathic refractory sideroblastic anemia.

Five of seven patients with idiopathic refractory sideroblastic anemia carried an HLA-A3 alloantigen (relative risk, 7.3; P = 0.02). The significance of this association was strengthened by study of two pedigrees. An abnormality in iron metabolism was found in two siblings who had an HLA-A3,B14 haplotype in common with the first proband. A second proband with idiopathic refractory sideroblastic...

The natural history of refractory idiopathic thrombocytopenic purpura.

Prognostic significance of thrombocytosis in idiopathic sideroblastic anemia.

In order to determine the prognostic significance of thrombocytosis in idiopathic sideroblastic anemia, the clinical courses of 17 patients were reviewed. Six patients (36%) had thrombocytosis, and none developed acute leukemia. Nine patients (53%) had normal platelet counts, and one developed acute leukemia. Two patients (12%) were thrombocytopenic, and one died of acute leukemia. There was li...

Prognostic value of platelet counts in idiopathic sideroblastic anemia.

Any compendium of facts and factors that can weight or cast doubt on the quantitative significance of serum fernitin measurement as a measure of iron stores is not complete without mention of immunologic nonidentical isofernitins. Liven on spleen fernitin is used as the refenence standard and for the production of antibody, yet there is abundant evidence that the ferritin in serum is immunologi...