Hemoglobin J-Korat Disease: A Case Report

Hemoglobin (Hb) J-Korat, or Hb J-Bangkok or Hb Meinung or Hb Manado is the abnormal hemoglobinresulted from the combination of 2 normal alpha and 2 abnormal beta globin chains whose glycine at the 56 th position is substituted by aspartic acid. It is transmitted as an autosomal recessive gene. Its heterozygote has noany clinical or hematological manifestation while its homozygote has never been mentioned, so far. Here wereport one case of Hb J-Korat homozygote or disease. She was a 48-year old woman who was referred to thehematologist because of progressive fatigue and chest discomfort due to anemia for one month. The physicalexamination revealed moderate pallor without jaundice, no thalassemic facy, no hepatosplenomegaly. She wasextensively investigated for finding the cause of anemia and finally she was definitely diagnosed to have the irondeficiency anemia, depending on the combination of hypochromic microcytic anemia, low serum ferritin and lowserum iron, with the underlying Hb J-Korat disease which was approved by the well trained medical technologist.After the oral iron therapy was accomplished within three months and also the metromenorrhagia which wassupposed to be the cause of iron deficiency, was corrected by the gynecologist, she was free from any symptom.But the laboratory tests showed she still had very mild degree of anemia (Hb 11.9 g%), normal red blood cellindices and normal RBC morphology which were supposed to be the manifestation of Hb J-Korat disease per se.