Bunyaratvej A, Fucharoen S, Butthep P, Sae-ung N, Kamchonwongpaisan S, Khuhapinant A
Department of Pathology, Faculty of Medicine, Ramathibodi Hospital, Bangkok, Thailand.
Southeast Asian J Trop Med Public Health. 1995;26 Suppl 1:257-60.
Two main types of thalassemia have been categorized according to defective production of the globin gene ie alpha-thalassemia and beta-thalassemia. We report different red cell abnormalities between these two types. The study included 139 thalassemic patients including 91 patients with hemoglobin (Hb) H disease (52 cases with the classical genotype and 39 cases with Hb Constant Spring) and 48 were beta-thalassemia/Hb E disease. The deformability index of thalassemic red cells measured by laser diffractometer was significantly lower than that of normal red cells. Increased susceptibility of the thalassemic red cells to monocyte phagocytosis was markedly noted. Few sialic acid molecules were scattered on red cell surface of thalassemic red cells. Reticulocytes with delayed maturation stage were also observed in thalassemia indicating enhanced release from the bone marrow. The alpha-thalassemic red cells had relatively better deformability, increased susceptibility to phagocytosis, reduced sialic acid content and greater degree irregular distribution of sialic acid on red cell surface as compared to beta-thalassemic red cells. The alpha-type with hemoglobin Constant Spring (Hb CS) had increased percentage of reticulocyte and young reticulocyte (high fluorescent intensity) as compared to beta-thalassemic red cells. The different abnormalities between alpha- and beta-thalassemic red cells may lead to different mechanism of red cell destruction and different severity of the disease.
根据珠蛋白基因产生缺陷，地中海贫血主要分为两种类型，即α地中海贫血和β地中海贫血。我们报告了这两种类型之间不同的红细胞异常情况。该研究纳入了139例地中海贫血患者，其中包括91例血红蛋白（Hb）H病患者（52例为经典基因型，39例为Hb Constant Spring）以及48例β地中海贫血/Hb E病患者。通过激光衍射仪测量的地中海贫血红细胞的变形性指数显著低于正常红细胞。明显观察到地中海贫血红细胞对单核细胞吞噬作用的敏感性增加。在地中海贫血红细胞表面散布的唾液酸分子较少。在地中海贫血中还观察到成熟阶段延迟的网织红细胞，这表明从骨髓中释放增加。与β地中海贫血红细胞相比，α地中海贫血红细胞具有相对较好的变形性、对吞噬作用的敏感性增加、唾液酸含量降低以及唾液酸在红细胞表面的不规则分布程度更高。与β地中海贫血红细胞相比，具有血红蛋白Constant Spring（Hb CS）的α型网织红细胞和年轻网织红细胞（高荧光强度）的百分比增加。α和β地中海贫血红细胞之间的不同异常可能导致红细胞破坏的不同机制和疾病的不同严重程度。
