Publication: Acute haemolytic crisis in a Thai patient with homozygous haemoglobin Constants Spring (Hb CS/CS): A case report
Issued Date
2004-12-01
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ISSN
02724936
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2-s2.0-11044237738
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Mahidol University
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SCOPUS
Bibliographic Citation
Annals of Tropical Paediatrics. Vol.24, No.4 (2004), 323-328
Suggested Citation
Vip Viprakasit, Gavivann Veerakul, Kleebsabai Sanpakit, Bunchoo Pongtanakul, Worrawut Chinchang, Voravarn S. Tanphaichitr Acute haemolytic crisis in a Thai patient with homozygous haemoglobin Constants Spring (Hb CS/CS): A case report. Annals of Tropical Paediatrics. Vol.24, No.4 (2004), 323-328. doi:10.1179/027249304225019145 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/21459
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Title
Acute haemolytic crisis in a Thai patient with homozygous haemoglobin Constants Spring (Hb CS/CS): A case report
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Abstract
Acute haemolysis associated with mild upper respiratory tract infection was observed in a Thai boy who presented with a rapid decline in haemoglobin (Hb) levels, haemoglobinuria and evidence of intravascular haemolysis. Several possible causes giving rise to such a condition were excluded including G6PD deficiency, which is extremely common in Thailand. Subsequent haematological and molecular analyses demonstrated that the patient was homozygous for Hb Constant Spring (Hb CS/CS), an α globin haemoglobinopathy. It has been shown previously that patients with homozygous Hb CS had mild haemolytic anaemia secondary to an accumulation of αCSchains, which are toxic to red blood cell membrane cytoskeletons. Increased body temperature might induce more precipitation of this ot globin variant. This report highlights the importance of Hb CS/CS as a potential predisposing cause of acute haemolysis in children that might be aggravated by acute bacterial or viral infections. This is particularly relevant for patients of Southeast Asian descent where this abnormal haemoglobin is highly prevalent. © 2004 The Liverpool School of Tropical Medicine.