Clinical diagnosis of sickle-C disease.

Abstract

In 1949, Pauling reported that the hemoglobin molecule in sickle-cell anemia differed from normal adult hemoglobin.1Subsequently, a number of abnormal hemoglobins have been described. Numerically, the most important of these abnormal hemoglobins are S- and C-hemoglobin. When the two are present in the same person, a characteristic syndrome may occur, which is designated as sickle-C disease. The C-hemoglobin is said to occur in 2% of the American Negro population, either alone or in combination with other hemoglobin types.2Thus it is not rare, and an area which has a large Negro population may expect to see disorders arising therefrom. Until recently, the pathogenic significance of the heterozygous combination of S- and C-hemoglobins was not recognized, and the sickle trait was regarded as a completely benign, inherited abnormality of the erythrocyte. Through the work of Pauling3and others it became possible to identify other abnormal hemoglobins in