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先天性红细胞生成异常性贫血——IV型。

Congential dyserythropoietic anemia--type IV.

作者信息

Benjamin J T, Rosse W F, Daldorf F G, McMillan C W

出版信息

J Pediatr. 1975 Aug;87(2):210-6. doi: 10.1016/s0022-3476(75)80581-6.

Abstract

Congenital dyserythropoietic anemia is characterized by ineffective erythropoiesis and increased numbers of multinucleated red cell precursors in the marrow. This syndrome has been subclassified on the basis of morphologic differences in the red cell precursors. Type I is characterized by megaloblastoid erythropoiesis and macrocytosis; Type II, by normoblastic multinuclearity and normocytosis; and Type III, by frequent giant multinucleated erythroblasts and macrocytes. Type II is further distinguished from the other types by serologic and ultrastructural abnormalities. The patient presented in this report does not fit any of the above categories; her red cells are similar to Type II congenital dyserythropoietic anemia cells, but no characteristic ultrastructural or serologic abnormalities are present. It is suggested that this patient may represent an additional variant of congenital dyserythropoietic anemia, Type IV.

摘要

先天性红细胞生成异常性贫血的特征是红细胞生成无效以及骨髓中多核红细胞前体数量增加。该综合征已根据红细胞前体的形态学差异进行了亚分类。I型的特征是类巨幼红细胞生成和大细胞性;II型的特征是正常幼红细胞多核化和正常红细胞性;III型的特征是频繁出现巨大的多核成红细胞和大红细胞。II型通过血清学和超微结构异常与其他类型进一步区分。本报告中呈现的患者不符合上述任何类别;她的红细胞与II型先天性红细胞生成异常性贫血细胞相似,但不存在特征性的超微结构或血清学异常。有人提出该患者可能代表先天性红细胞生成异常性贫血的另一种变体,即IV型。

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