Clinical course and molecular characterization of a compound heterozygote for sickle hemoglobin and hemoglobin kenya

We describe a 25‐year‐old black woman who presented with a long history of anemia requiring transfusions during childhood and adolescence. Molecular analysis revealed her to be a compound heterozygote for the sickle mutation and the ∼22.7 kb deletion associated with hemoglobin Kenya. This patient's clinical course was more severe than previously reported for the Hb S/Hb Kenya genotype, a probable consequence of concomitant iron deficiency. © 1992 Wiley‐Liss, Inc.