Publication: Pyruvate kinase deficiency in an alpha-thalassemia family: First case report in Thailand
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1997-12-01
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01251562
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2-s2.0-0031306116
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Mahidol University
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SCOPUS
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Southeast Asian Journal of Tropical Medicine and Public Health. Vol.28, (1997), 64-68
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Voravarn S. Tanphaichitr, Vinai Suvatte, Chularatana Mahasandana, Gavivann Veerakul, Parichat Pung-amritt, Kalya Tachavanich, Hiroshi Ideguchi Pyruvate kinase deficiency in an alpha-thalassemia family: First case report in Thailand. Southeast Asian Journal of Tropical Medicine and Public Health. Vol.28, (1997), 64-68. Retrieved from: https://repository.li.mahidol.ac.th/handle/123456789/18055
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Pyruvate kinase deficiency in an alpha-thalassemia family: First case report in Thailand
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In Thailand, the most common cause of chronic hemolytic anemia is thalassemia hemoglobinopathy. We report here a 10-year-old girl with pyruvate kinase. (PK) deficiency who was initially diagnosed to have Hb H disease, like her sister. The patient had a history of neonatal jaundice which required blood exchange transfusion twice and phototherapy. She became anemic and regular blood transfusion was required since the age of 2 1/2 months. She was very anemic compared to her sister and was transfusion dependent. Besides, she never had red cell inclusion bodies, thus re-evaluation was performed The diagnosis of red cell pyruvate kinase deficiency and the exclusion of Hb H disease was achieved after cessation of blood transfusion for 3 months The family study also confirmed the diagnosis. The patient is now on high transfusion and iron chelation. She is doing well with mild splenomegaly.