Interaction between HBS-beta-o-thalassemia and alpha-thalassemia.
We have defined the clinical and laboratory characteristics of a group of patients with HbS-beta o-thalassemia plus alpha-thalassemia, by analysis of erythrocyte indices, hemoglobin A2 and F levels, globin biosynthesis studies and alpha-globin gene mapping. Patients with HbS-beta o + alpha-thalassemia closely resembled individuals with HbS-beta o-thalassemia except for balanced globin synthesis ratios and a lower HbF level. The frequency of painful crises, leg ulceration, aseptic necrosis of bone and acute chest syndrome was similar in HbS-beta o + alpha-thalassemia patients and controls with sickle cell anemia (HbSS), HbSS-alpha-thalassemia and HbS-beta o-thalassemia. These findings are consistent with previous work which failed to show a reduction in the vaso-occlusive severity of sickle cell disease by the coexistence of alpha-thalassemia.
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D004588:Electrophoresis, Cellulose Acetate; D005260:Female; D005319:Fetal Hemoglobin / Q000032:analysis; D005838:Genotype; D005914:Globins / Q000096:biosynthesis; D006443:Hemoglobin A2 / Q000032:analysis; D006451:Hemoglobin, Sickle / Q000032:analysis; D006801:Humans; D008297:Male; D013789:Thalassemia / Q000097:blood* / Q000503:physiopathology
D006451:Hemoglobin, Sickle; D005914:Globins; D006443:Hemoglobin A2; D005319:Fetal Hemoglobin