Blood, 1958, Vol. 13, No. 6, pp. 559-568.
© 1958 American Society of Hematology, Inc.
An Unusual Type of Hemoglobinopathy Resembling Sickle
Cell-Thalassemia Disease in a Jamaican Family
L. N. WENT 1 and
J. E. MACIVER 1
1 Department of Pathology, University College of the West Indies, Jamaica.
Three generations of a Jamaican family of African extraction are described,
in several members of which an abnormal gene is carried. This gene produces
high levels of fetal hemoglobin unassociated with the usual stigmata of
thalassemia. It is found in all three generations of the family associated with
hemoglobin A only and is also found in at least two members of the family
interacting with hemoglobin S. In the latter combination little or no disability
results.
The mode of inheritance of this abnormal gene is discussed, and reasons
are put forward for a possible protective effect of high fetal hemoglobin
levels due to inhibition of sickling.
The findings in the cord blood of the youngest child, including an unusually high percentage of sickling, are discussed, together with follow-up
studies to the age of 25 weeks.
Submitted on July 23, 1957
Accepted on December 9, 1957
