Studies in Thalassaemia

Haemoglobin-H Disease

“…The life span of red cells appears to be variable, as a patient who at one time showed clear evidence of haemolysis later had an almost normal cell survival. These findings are similar to those in thalassaemia (Bailey and Prankerd, 1958), which the disease closely resembles in clinical features and haematological findings. However, it differs from thalassaemia minor in that HbA2 is not increased in the patients or their relatives (Vella, Wells, Ager, and Lehmann, 1958).…”

The Distribution of Hemoglobin Types in Thalassemic Erythrocytes*

“…This variability, evident at mere examination of red cell morphology, has been supported by the early studies on red cell survival, which showed that the red cell population contains very short-living as well as rather longliving cells (1). Essentially similar findings were obtained in some studies by using chromated erythrocytes (2,3); in other studies no initial rapid fall of the chromium survival curve was observed, but there was indirect evidence compatible with the presence of a very short-lived population of red cells (4). Support for heterogeneity has also been obtained by the technique for the histochemical demonstration of hemoglobin F (5), all reports agreeing on the nonuniform distribution of fetal hemoglobin over the red cell population (6)(7)(8).…”

The Turnover of Hemoglobins A, F, and A2 in the Peripheral Blood of Three Patients With Thalassemia*

“…thesis in thalassemia. Large quantities of hemoglobin are apparently produced, but undergo premature destruction in the marrow (ineffective erythropoiesis) (4)(5)(6)(7)(8)(9). Most of the hemoglobin released into the peripheral blood does not usually achieve a normal survival.…”