Bunyaratvej A, Fucharoen S, Greenbaum A, Mohandas N
Department of Pathology, Ramathibodi Hospital and Thalassemia Center, Faculty of Medicine, Mahidol University, Bangkok, Thailand.
Am J Clin Pathol. 1994 Aug;102(2):217-22. doi: 10.1093/ajcp/102.2.217.
Differences in the pathobiologic features of alpha and beta thalassemic red cells include differing derangements in the ability of these cells to regulate their volume. To explore differences in cell hydration in these two red cell phenotypes, heterogeneity in hemoglobin concentration of individual red cells was quantitated. Red cells from 91 patients with alpha thalassemia (hemoglobin H disease) and 69 patients with beta thalassemia of the genotype beta-thal zero/Hb E were analyzed. Marked differences between these thalassemias were noted in the heterogeneity of hemoglobin concentration among individual cells. Decreased cell hemoglobin concentration and increased cell hydration were features of alpha-thalassemic blood samples, whereas both decreased and increased cell hemoglobin concentration as a result of cell hydration and dehydration, respectively, were features of beta-thalassemic blood samples. The documented differences in the hemoglobin concentration distributions should prove useful in distinguishing between the two thalassemic phenotypes.
α和β地中海贫血红细胞的病理生物学特征差异包括这些细胞调节其体积能力的不同紊乱。为了探究这两种红细胞表型在细胞水合作用方面的差异，对单个红细胞血红蛋白浓度的异质性进行了定量分析。分析了91例α地中海贫血（血红蛋白H病）患者和69例基因型为β0/βE的β地中海贫血患者的红细胞。在这些地中海贫血中，单个细胞间血红蛋白浓度的异质性存在显著差异。细胞血红蛋白浓度降低和细胞水合作用增加是α地中海贫血血样的特征，而细胞水合作用和脱水分别导致细胞血红蛋白浓度降低和增加则是β地中海贫血血样的特征。所记录的血红蛋白浓度分布差异应有助于区分这两种地中海贫血表型。
