Anemia and hydrops in a fetus with homozygous hemoglobin constant spring

• Main Authors: Pimlak Charoenkwan, Supatra Sirichotiyakul, Pharuhas Chanprapaph, Fuanglada Tongprasert, Rawee Taweephol, Rattika Sae-Tung, Torpong Sanguansermsri
• Format: Journal
• Published: 2018
• Subjects: Medicine
• Online Access: https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=33845686816&origin=inward; http://cmuir.cmu.ac.th/jspui/handle/6653943832/61796
• 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.