Hemoglobin H Disease with Hemoglobin J-Korat: Report of Two Cases
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Abstract: Hemoglobin J-Korat or J-Bangkok (αA2β56gly→asp2) has been occasionally reported in Thailand in the forms of trait or double or triple heterozygotes. Here we report 2 unrelated cases of triple heterozygotes of Hb H, alpha(0)- and alpha(+)-thalassemias with Hb J-Korat traits. They are 55- and 50-year-old Thai patients having moderate pallor, spleen enlargement 2-3 cm below left costal margin, no hepatomegaly. Their Hb concentrations ranges between Hb 8.7-9.6 g%, Hct 27.6-33.3 %, MCV 54.0-93.6 fL, MCH 15.7-29.5 pg, MCHC 28.9-31.5 g%, RDW 15.5-25.1 %, comparable to Hb 8.1 g%, Hct 29.5%, MCV 63.0 fL, MCH 17.3 pg with mild jaundice, mild hepato-splenomegaly of naïve Hb H disease. The diagnosis of this entity depends on the Hb electrophoresis that demonstrates the band of Hb J-Korat as well as Hb A and it could be confirmed by the genotype study. It seems that the patients with the triple heterozygous Hb H with Hb J-Korat trait have the clinical and hematological manifestations which are not different from that of Hb H disease per se. Furthermore, Hb H disease with Hb J-Korat trait still has acute hemolytic crisis during the exposure to the oxidative stress, the important characteristic of Hb H disease per se.
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