Anemia and hydrops in a fetus with homozygous hemoglobin constant spring

• Main Authors: Charoenkwan P., Sirichotiyakul S., Chanprapaph P., Tongprasert F., Taweephol R., Sae-Tung R., Sanguansermsri T.
• Format: Article
• Language: English
• Published: 2014
• Online Access: http://www.scopus.com/inward/record.url?eid=2-s2.0-33845686816&partnerID=40&md5=50ebd4cef02d134adcac5c50054b50ab; http://www.ncbi.nlm.nih.gov/pubmed/17164653; http://cmuir.cmu.ac.th/handle/6653943832/1985
• Institution: Chiang Mai University

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.