Publication: Anemia and hydrops in a fetus with homozygous hemoglobin constant spring
Issued Date
2006-12-01
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ISSN
15363678
10774114
10774114
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2-s2.0-33845686816
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Mahidol University
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SCOPUS
Bibliographic Citation
Journal of Pediatric Hematology/Oncology. Vol.28, No.12 (2006), 827-830
Suggested Citation
Pimlak Charoenkwan, Supatra Sirichotiyakul, Pharuhas Chanprapaph, Fuanglada Tongprasert, Rawee Taweephol, Rattika Sae-Tung, Torpong Sanguansermsri Anemia and hydrops in a fetus with homozygous hemoglobin constant spring. Journal of Pediatric Hematology/Oncology. Vol.28, No.12 (2006), 827-830. doi:10.1097/01.mph.0000243662.56432.37 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/23445
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Title
Anemia and hydrops in a fetus with homozygous hemoglobin constant spring
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Abstract
Hemoglobin Constant Spring (Hb CS) is an unstable hemoglobin (Hb) variant that results from a nucleotide substitution at the termination codon of the α2-globin gene. The compound heterozygosity of α-thalassemia and Hb CS (-/αα) results in a Hb H/CS disease which is clinically more severe than deletional Hb H disease. Homozygosity of Hb CS (αα/ αα) is generally characterized with mild hemolytic anemia, jaundice, and splenomegaly. The authors report 1 case with Hb CS homozygosity who presented with fetal anemia and hydrops. Intrauterine transfusions were given which rendered a favorable outcome. This report demonstrates an unusual and serious in utero complication in a fetus with Hb CS/CS. © 2006 Lippincott Williams & Wilkins, Inc.