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两例II型先天性红细胞生成异常性贫血的超微结构特征

Ultrastructural features in two cases of congenital dyserythropoietic anemia type II.

作者信息

Moldoveanu E, Coliţă A, Hălălău F

机构信息

Victor Babeş Institute, Bucharest, Romania.

出版信息

Rom J Morphol Embryol. 1994 Jul-Dec;40(3-4):133-6.

PMID:7548885
Abstract

Two patients with congenital dyserythropoietic anemia (CDA) type II were investigated in bone marrow as well as in blood cells. The majority of red cell precursors showed typical morphological abnormalities: high incidence of binucleated erythroblasts, normoblastic hyperplasia, nucleo-cytoplasmic dissociations, karyorrhexis of some nuclei, presence of synartesis phenomenon, invagination of cytoplasmic portions into the nuclear area and presence of marginal cisterna. The modified erythroblasts were present in the peripheral blood, too. The presence of Gaucher-like cells suggests an abnormal catabolism of the red cell precursors membranes.

摘要

对两名II型先天性红细胞生成异常性贫血(CDA)患者的骨髓和血细胞进行了研究。大多数红细胞前体表现出典型的形态学异常:双核成红细胞发生率高、正成红细胞增生、核质解离、部分细胞核核溶解、存在联会现象、细胞质部分内陷至核区域以及存在边缘池。在外周血中也存在形态改变的成红细胞。高雪样细胞的存在提示红细胞前体细胞膜的分解代谢异常。

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