Transplantation of Cord-Blood Stem Cells into a Patient with Severe Thalassemia

Abstract

Several different kinds of thalassemia and hemoglobinopathy are prevalent in Southeast Asia.^1,2 The frequency of the α-thalassemias reaches 30 to 40 percent in northern Thailand, whereas the β-thalassemias occur at a frequency of 3 to 9 percent. Hemoglobin E, the hallmark hemoglobinopathy of Southeast Asia, occurs at a frequency of 50 to 60 percent at the junction of Thailand, Laos, and Cambodia. Mutation and gene interaction account for more than 60 different clinical syndromes. Of these, homozygous β-thalassemia and hemoglobin E –β-thalassemia disease are the most common and the most severe clinical syndromes compatible with live birth. Hemoglobin E–β-thalassemia disease is more frequent than homozygous β-thalassemia in Southeast Asia because of the much higher frequency of hemoglobin E.