The First Observation of Homozygous Hemoglobin S . Alpha Thalassemia Disease and Two Types of Sickle Cell

S ICKLE CELL-THALASSEMIA DISEASE is known to be a severe or moderately severe type of congenital hemolytic anemia. The clinical and hematologic pictures of this hemoglobinopathy are usually similar to those found in the intermediate type of Cooley’s anemia; however, mild or asymptomatic cases have been reported by various investigators. 6 Singer et al.1 have described four Negro patients with mild sickle-cell thalassemia disease. In one of the patients, all hematologic indices including the MCV were within norma! limits. Aksoy and Lehmann and later Aksoy reported a total of 11 cases of sickle cell-thalassemia disease among Eti-Turks.45 Also in this community, five individuals were discovered who were heterozygous for thalassemia and hemoglobin S genes and they were neither anemic nor symptomatic.5 In sickle cell-thalassemia disease, the amount of hemoglobin S is highly variable, but it is usually more than 50 per cent. Silvestroni and his associates7 have reported that high values of hemoglobin S ranging between 67.9 and 97.1 per cent are constantly found in this hemoglobinopathy. NeeI2 reported two families in which both genes appeared to be present and hemoglobin analyses revealed that the patients had low hemoglobin A fractions, 22.3 and 36.2 per cent respectively. Aksoy5 also noticed that in two of his patients the