Blood, 1953, Vol. 8, No. 3, pp. 270-275.
© 1953 American Society of Hematology, Inc.
Studies on Abnormal Hemoglobins
VI. Electrophoretic Demonstration of Type S (Sickle Cell) Hemoglobin in Erythrocytes Incapable of Showing the Sickle Cell
Phenomenon
KARL SINGER M.D.1 and
BEN FISHER M.D.1
1 Department of Hematologic Research, Medical Research Institute, Michael
Reese Hospital, Chicago, Ill.
1. A family is reported in which 2 children have unmistakable sickle cell
anemia, but the mother’s red cells do not sickle; the father and a third child
exhibit the typical sickle cell trait. Electrophoretic studies of the hemoglobin
solution prepared from the mother’s erythrocytes demonstrated, however, the
presence of a small amount (5 per cent) of type S hemoglobin. This abnormal
component was identified by its mobility and by addition experiments.
2. The finding that the mother’s erythrocytes contain small quantities of S
hemoglobin, but are incapable of sickling, confirms the genetic concept that
sickle cell anemia will only develop when both parents transmit the gene for S
hemoglobin.
3. The physico-chemical and possible medico-legal aspects of the fact that S
hemoglobin is sometimes only detectable by means of electrophoresis, are discussed.
Submitted on August 5, 1952
Accepted on September 10, 1952
