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.
SCHELL NB, McGINLEY JM. SICKLE-CELL-HEMOGLOBIN-C DISEASE: Report of a Case with Electrophoretic Studies of Hemoglobin in Family Members. AMA Am J Dis Child. 1956;91(1):38–44. doi:https://doi.org/10.1001/archpedi.1956.02060020040008
Customize your JAMA Network experience by selecting one or more topics from the list below.
Create a personal account or sign in to: