SICKLE CELL-HEMOGLOBIN D DISEASE*

doi:10.1059/0003-4819-50-1-94

SICKLE CELL-HEMOGLOBIN D DISEASE*

ERNEST W. SMITH†; and
C. LOCKARD CONLEY, F.A.C.P.

Requests for reprints should be addressed to Ernest W. Smith, M.D., Assistant Professor of Medicine, The Johns Hopkins Hospital,
Baltimore 5, Maryland
.

Excerpt

Soon after the discovery that sickle (S) hemoglobin can be differentiated from normal (A) hemoglobin by electrophoresis,¹ other abnormalities of hemoglobin were recognized. In studying a white patient who previously had been thought to have sickle cell anemia, Itano² found that the erythrocytes of the mother could not be made to sickle, although the electrophoretic pattern was indistinguishable from that of sickle cell trait. The abnormal hemoglobin which did not produce sickling also differed from S hemoglobin because of its normal solubility in the reduced state, and was subsequently designated as hemoglobin D. The atypical hemolytic disorder in the patient

This 100-word excerpt has been provided in the absence of an abstract.

Summario in Interlingua

Le presentia de morbo a cellulas falciforme e hemoglobina D esseva demonstrate in un masculo de racia blanc in Nord-Carolina. Su curso clinic esseva characterisate per anemia, recurrentia de jalnessa, dolores in le extremitates e in le dorso, e repetite episodios de pneumonitis. Falciformation de su erythrocytos poteva esser evocate, e le patrono electrophoretic de su hemoglobina in papiro-filtro a pH 8,6 non esseva distinguibile ab illo de anemia a cellulas falciforme. Tamen, in plattas de agar a pH 6,2, il occurreva un clar separation del componentes S e D, e un plus micre componente con le mobilitate de hemoglobina fetal esseva etiam demonstrate. Esseva constatate que le matre del patiente e cinque de su consanguineos habeva le tracto de hemoglobina D. Le patre del patiente e sex de su consanguineos habeva le tracto de cellulas falciforme. Nulle evidentia de ancestria negre existeva in le lineage materne o in le lineage paterne. Le datos genetic que esseva obtenite supporta le opinion que hemoglobina D es un allel del hemoglobinas A, C, e S.

Article and Author Information

↵* Received for publication October 28, 1957.
From the Department of Medicine, The Johns Hopkins University School of Medicine and Hospital.
↵† Department of Medicine of the Howard Hughes Medical Institute.
This investigation was supported in part by a Research Grant of the Division of Research Grants and Fellowships, National Institutes of Health.