HEMOGLOBIN S-J-BANGKOK DISEASE: A NEWLY IDENTIFIED SICKLING DISORDER

Abstract

Several members of a Negro family from Southern Illinois were found to be heterozygous for Hb J-Bangkok (α₂^Aβ₂⁵⁶ gly→asp), a rare hemoglobin abnormality previously reported only in individuals of Thai or Chinese ancestry. In 2 children (ages 3 and 8) Hb J-Bangkok was present in combination with sickle hemoglobin. Both children exhibited mild hypochromic, microcytic anemia, apparently due to iron deficiency. Neither child demonstrated hemolytic disease, enlargement of the liver or spleen, or symptomatic sickle crises. Hb J comprised 54-59% of the total Hb in all family members having this Hb, in common with previous reports of this variant. Hb A₂ and alkali-resistant Hb were present in normal concentrations. The interaction between sickle Hb and the Hb J in the doubly heterozygous subjects was evaluated by measurement of minimum gelling concentrations of deoxygenated mixtures of the two Hbs. Mixtures containing 40% sickle hemoglobin gelled at 32.5-33.7 gm./dl., values similar to those obtained with a 40:60 mixture of Hbs S and A. The pathologic potential of the combination of Hbs S and J-Bangkok therefore appears to be comparable to sickle cell trait.

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