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Two unrelated families are described which include two heterozygotes for Hb Stanleyville II, two compound heterozygotes for Hb Stanleyville II and Hb S, and one compound homozygote for the two abnormal hemoglobins. The homozygote did not produce normal alpha chains, the abnormal chains accounted for 32-35% of the total alpha chain of four heterozygotes, and the alpha/non-alpha synthetic ratios were in the range 0.59-0.77. These data demonstrate the presence of an alpha-thalassemia gene linked to the alpha-Stanleyville II mutation.
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