Blood, 1960, Vol. 16, No. 3, pp. 1239-1252.
© 1960 American Society of Hematology, Inc.
Congenital Hemolytic Disease Associated with Red Cell
Inclusion Bodies, Abnormal Pigment Metabolism
and an Electrophoretic Hemoglobin Abnormality
J. L. SCOTT 1,
A. HAUT 1,
G. E. CARTWRIGHT 1, and
M. M. WINTROBE 1
1 Department of Medicine, University of Utah College of Medicine, Salt Lake
City, Utah.
1. A fourth case of the syndrome of congenital hemolytic anemia with abnormal pigment metabolism and red cell inclusion bodies following splenectomy is described.
2. In this case an abnormality was found on paper and starch electrophoresis of the red cell hemolysate at pH 8.6. A similar abnormality has not
been reported previously.
3. An increased rate of erythrocyte autohemolysis was found during in vitro
incubation, Partial correction of this defect occurred in the presence of
glucose or purine ribosides.
4. Genetic transmission of the defect could not be demonstrated.
Submitted on February 10, 1960
Accepted on April 18, 1960
