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January 1956

SICKLE-CELL-HEMOGLOBIN-C DISEASE: Report of a Case with Electrophoretic Studies of Hemoglobin in Family Members

Author Affiliations

U.S.N.R.; U.S.N.R.
From the Pediatric Service of U. S. Naval Hospital, St. Albans, N. Y.

AMA Am J Dis Child. 1956;91(1):38-44. doi:10.1001/archpedi.1956.02060020040008

THE ENTITY of sickle-cell-hemoglobin-C disease is not a rarity. After Pauling and co-workers1 had first demonstrated a unique electrophoretic pattern for sickle-cell hemoglobin, Itano and Neel2 used this technique in 1950 to ascertain a new inherited abnormality of hemoglobin. This was initially designated hemoglobin III3 and subsequently was renamed hemoglobin C.4

Since that time, 71 cases of the syndrome of sickle-cell-C-hemoglobin disease have been described in the English language literature.* Undoubtedly many more cases of this combination have been overlooked, for the incidence of hemoglobin C has been estimated to be between 0.3% and 3% of the American Negro population.†

It is important for the pediatrician to remain aware of this disease, for it mimics sickle-cell anemia but differs in prognosis and management. The following case report illustrates the resemblance to classical sickle-cell anemia and the necessity for performing electrophoretic hemoglobin studies to distinguish the two.

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