Blood, 1959, Vol. 14, No. 7, pp. 816-827.
© 1959 American Society of Hematology, Inc.
Multiple Inherited Erythrocyte Abnormalities in an
American Negro Family: Hereditary Spherocytosis,
Sickling and Thalassemia
FLOSSIE COHEN 1,
WOLF W. ZUELZER 1,
JAMES V. NEEL 1, and
ABNER R. ROBINSON 1
1 Child Research Center of Michigan, The Children’s Hospital of Michigan,
Detroit, and The University of Mchigan, Ann Arbor.
An American Negro family has been described in which there are found
the genes responsible for three inherited abnormalities of the erythrocyte,
namely, the genes resulting in spherocytosis, a thalassemia-like trait and
the sickling phenomenon. The study of this family provides evidence for the
independent segregation of the genes responsible for this type of thalassemia
and the sickling phenomenon, and for spherocytosis and the sickling phenomenon. It is noteworthy that the thalassemia gene present in this family is not
associated with an increased proportion of hemoglobin A2, and fails to exhibit
"factor interaction" with the sickle cell gene, and the question is raised whether
these attributes characterize a type of thalassemia gene non-allelic to the
sickle cell gene.
Submitted on August 8, 1958
Accepted on January 12, 1959
