地中海贫血红细胞系超微结构改变的研究

ULTRASTRUCTURE STUDIES ON ERYTHROID LINE IN THALSSEMAIA

本文报道了6例地中海贫血红细胞系列的超微结构改变,包括中、晚幼红细胞,网织红细胞及部份成熟红细胞的胞浆及胞核内可见电子密度较高的包涵体(α或β肽链的沉积);核膜有缺失或重叠,核周间隙扩大;各期红细胞中易见自噬泡和空泡形成;红细胞外形变化显著,发现有特征性的棒形红细胞。此外,还观察到红细胞在狭窄的脾窦中被扣押、挤压、破坏的现象。

Ultrastructure varities in erythroid line of 6 patients with Thalassemia were studied by electron microscopy. A number of electron-dense inclusions (alpha or beta chains precipitation) were noted in cytoplasm or nuclei of polychromatic erythroblasts, normoblasts, reticulocytes and erythrocytes. The nuclear membranes showed absence or duplication and the space between two layers of nuclear membranes dilated. Autophage vacuoles and vacuolation were easily observed within the early and late stages of erythroid series. The figure of red cells was markedly abnormal, the characteristic ropalocytes might be found. In addition, the various forms of squeezed and partly destructed RBC which had been sequestrated in the minute sinuses of spleen were observed. The observations above provide direct morpdological evidences of the pathogenesis in Thalassemia