Abstract
The 1st case of hemoglobin H disease in Eti-Turks, an ethnic group living in Southern Turkey, is presented. The family study of a 23 year old man showed that he was homozygous for alpha-thalassemia. The mode of inheritance of hemoglobin H disease is discussed and the compatibility of homozygous alpha-thalassemia with life is stressed. The hypothesis advanced by Ingram and Stretton which states that hemoglobin H disease might be a manifestation of homozygous alpha-thalassemia is favored. To overrule many objections raised against this hypothesis the heterogeneity of alpha-thalassemia genes is accepted.