Dell'Orbo C, Marchi A, Quacci D
Department of Citomorphology, University of Cagliari, Italy.
Histol Histopathol. 1992 Jan;7(1):7-10.
The ultrastructural findings of erythroblasts and reticulocytes in one case of congenital dyserythropoiethic anemia (CDA) associated with a haemoglobinopathy, sickle cell beta thalassemia minor (Type V CDA), is described. The observations can be summarized as follows: 1) A lot of large breaks are present in the erythroblast nuclear envelope. 2) Nuclear membrane evaginations are filled with dense loose chromatin. 3) Electron-transparent areas (moth eaten chromatin) are evident in dense chromatin. 4) Electron-dense granular material, related to altered haemoglobin chain storage, is evident in the nucleus and in the cytoplasm. 5) Iron deposits are present in mitochondrial matrix. 6) Myelinic figures are present in reticulocyte cytoplasm. For the first time the ultrastructural findings in this type of associated CDA are described and related to the double origin of clinical symptoms.
本文描述了一例与血红蛋白病——镰状细胞β地中海贫血轻型（Ⅴ型先天性红细胞生成异常性贫血）相关的先天性红细胞生成异常性贫血（CDA）患者的成红细胞和网织红细胞的超微结构发现。观察结果总结如下：1）成红细胞核膜出现大量大的断裂。2）核膜内陷充满致密疏松的染色质。3）致密染色质中可见电子透明区（虫蚀状染色质）。4）与血红蛋白链储存改变相关的电子致密颗粒物质在细胞核和细胞质中可见。5）线粒体基质中有铁沉积。6）网织红细胞细胞质中有髓鞘样结构。首次描述了这种类型的相关CDA的超微结构发现，并将其与临床症状的双重起源相关联。
