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骨髓和脾脏中存在成红细胞多核现象。具有先天性异常红细胞生成性贫血样异常,但无红细胞生成异常的功能证据。

Erythroblast multinuclearity in bone marrow and spleen. Congenital dyserythropoietic anemia-like abnormalities without functional evidence of dyserythropoiesis.

作者信息

Lillicrap D P, Corbett W E, Giles A R, Ginsburg A D

出版信息

Arch Pathol Lab Med. 1987 Jun;111(6):536-9.

PMID:3579510
Abstract

A dyserythropoietic syndrome with coincidental immune thrombocytopenia seen during pregnancy is discussed. The morphological and serological results in this case most resemble type II congenital dyserythropoietic anemia. However, functional evidence of dyserythropoiesis was absent and the patient was not anemic. Splenectomy was performed for the resistant thrombocytopenia and microscopic examination of the spleen showed evidence of extramedullary dyserythropoiesis. The significance of these findings is discussed with regard to the spectrum of recognized dyserythropoietic disorders and the unusual discrepancy between the abnormal morphology and the absence of functional dyserythropoiesis.

摘要

本文讨论了一例在孕期出现的伴有免疫性血小板减少症的异常红细胞生成综合征。该病例的形态学和血清学结果与II型先天性异常红细胞生成性贫血最为相似。然而,该病例缺乏异常红细胞生成的功能证据,且患者并无贫血症状。因血小板减少症难以控制,故实施了脾切除术,脾脏的显微镜检查显示存在髓外异常红细胞生成的证据。结合已确认的异常红细胞生成性疾病谱以及异常形态与缺乏功能性异常红细胞生成之间的异常差异,对这些发现的意义进行了讨论。

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