The relationship between anemia, fecal stercobilinogen, erythrocyte survival, and globin synthesis in heterozygotes for beta-thalassemia

• Published in: Blood Vol. 46; no. 5; pp. 693 - 698
• Main Authors: Gallo, E, Pich, P, Ricco, G, Saglio, G, Camaschella, C, Mazza, U
• Format: Journal Article
• Language: English
• Published: 01.11.1975
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood.V46.5.693.bloodjournal465693

Erythrocyte survival, fecal stercobilinogen, and the alpha/beta-chain synthesis ratio in recticulocytes and bone marrow (six cases only) were determined and related to the hemoglobin levels in ten heterozygous carriers of beta-thalassemia with different degrees of anemia. Erythrocyte survival values were low, though not related to the degree of anemia, whereas stercobilinogen values and changes in the peripheral blood alpha/beta-chain ratios were so correlated. In the case of bone marrow, on the other hand, the ratio between alpha-chain and beta-chain synthesis was virtually 1, irrespective of the degree of anemia. It is suggested that the severity of anemia in heterozygous carriers is independent of peripheral hemolysis and entirely attributable to destruction of erythroblasts within the marrow.