Am J Hematol. 1992 Dec;41(4):289-91.

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

Waye JS, Cai SP, Eng B, Chui DH, Francombe WH.

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Provincial Hemoglobinopathy DNA Diagnostic Laboratory, McMaster University Medical Centre, Hamilton, Ontario, Canada.

Abstract

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 approximately 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.

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