Publication: Pyruvate kinase deficiency in an alpha-thalassemia family: First case report in Thailand
Issued Date
1997-12-01
Resource Type
ISSN
01251562
Other identifier(s)
2-s2.0-0031306116
Rights
Mahidol University
Rights Holder(s)
SCOPUS
Bibliographic Citation
Southeast Asian Journal of Tropical Medicine and Public Health. Vol.28, (1997), 64-68
Suggested Citation
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/20.500.14594/18055
Research Projects
Organizational Units
Authors
Journal Issue
Thesis
Title
Pyruvate kinase deficiency in an alpha-thalassemia family: First case report in Thailand
Abstract
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.