Alpha-chain Thalassemia and Hydrops Fetalis in Malaya: Report of Five Cases By LIR-INJo LUAN ENG

identical with Fessas and Papaspyrou hemoglobin-has been found by the author to occur in 3.3 per cent Chinese newborn infants in Indonesia. The incidence is much lower in Indonesian newborns, about 0.4 per cent. This hemoglobin disappears during the first months of life, and is therefore not found in adults. “Bart’s ” hemoglobin has only rarely been found in disease states, and the author has found it in two cases of chronic hemolytic anemia ( to be published). Because this hemoglobin is not rare in healthy newborns, it may be questioned why it is not found more often in disease conditions. Assuming that the patients die too early to be detected, we have therefore screened diseased and stillborn babies in an attempt to correlate this hemo-globin with disease and fetal death. A correlation was indeed found between the presence of a large amount of this hemoglobin and a well defined type of clinical picture. Four cases of severe hydrops and erythroblastosis fetalis have been reported in association with “Bart’s ” hemoglobin from Indonesia.24 Studying the incidence of this fast moving hemoglobin in newborns in Ma-laya, the author, in collaboration with Dr. Ti,5 found an even higher incidence, 5.1 per cent, of this component in Chinese newborns in Kuala Lumpur. Exam-ining stillborn and diseased fetuses in this area, she again found five cases of severe hydrops and erythroblastosis in association with this hemoglobin. It has been postulated,4’6 that these cases of hydrops fetalis might be in-stances of homozygous a-chain thalassemia, based on the theory of Ingram and Stretton that in thalassemia, either the a-chain or the fl-chain production of hemoglobin might be impaired. This paper is a clinical and hematologic report on the Malayan cases. METHOD AND MATERIALS Routine hematologic examinations were carried out according to standard methods. Paper electrophoretic analysis of hemoglobins was carried out by the method of Smith and Conley,7 using veronal buffer at pH 8.6, ionic strength 0.06, and phosphate buffer, pH 6.5. Chromatographic study was done by the method described by Huisman and Jonxis, using ion-exchange resin IRC50.8 Alkali-resistant hemoglobin was examined by the method of Singer, Chernoff an