Sickle Cell Syndromes. III. Silent-Carrier α-Thalassemia in Combination with Hemoglobin S and Hemoglobin C

Intraerythrocytic Hemoglobin Crystals in Sickle Cell-hemoglobin C Disease.

Hemoglobin S gelation and sickle cell disease.

By William A. Eaton and James Hofrichter T HE FUNDAMENTAL cause ofsickle cell disease is the decreased deformability of the sickled red cell produced by gelation of hemoglobin S. Partial inhibition of gelation should therefore reduce clinical severity, while complete inhibition should result in a “cure.” These basic ideas have stimulated an enormous effort to understand the gelation process in ...

Heterozygote Hemoglobin J Iran in Combination with Hemoglobin H Disease

This is a report concerning a concurrent case of hemoglobin J Iran (Hb J Iran) and Hemoglobin H (Hb H) disease in an Iranian woman. The patient was coincidentally found during the course of routine pre-marital genetic counselling for her son. The diagnosis of heterozygote Hb J Iran for her son, ultimately led to the diagnosis of concurrent Hb J Iran and Hb H disease. The hematological examinati...

Thalassemia-hemoglobin C disease; a new syndrome presumably due to the combination of the genes for thalassemia and hemoglobin C.

IN PREVIOUS CO? -IMUNICATIONS’-3 the hematologic effects of the gene for an abnormal hemoglobin, since designated as hemoglobin C, were described in two combinations: A-C, an asymptomatic carrier state in which the hemoglobin consists of a mixture of A (normal adult type) hemoglobin amid C; secondly S-C, a variant of sickle cell disease in which sickle hemoglobin S is produced together with hem...

Red cell life span in sickle cell-hemoglobin C disease with a note about sickle cell-hemoglobin O ARAB.

Red cell survival was measured in ten subjects with S-C disease and one with S-O Arab (alpha 2 beta 2-121 glu yields lys) disease using both DF32p and 51Cr as tags. Red cell volume was slightly reduced in most patients (87% plus or minus 20% of predicted normal). In nine SC patients, mean red cell life (DF32p) was 28.9 plus or minus 4.0 days. For one SC subject it was significantly longer (47.9...