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播散性朗格汉斯细胞组织细胞增多症:一例光镜及超微结构研究的病例报告

Disseminated Langerhans cell histiocytosis: a case report with a light microscopic and ultrastructural study.

作者信息

Ionescu N, Toma C, Ciofu E, Popescu M, Lascu V

机构信息

Department of Cell Biology and Histology, Carol Davila, University of Medicine and Pharmacy, Bucharest.

出版信息

Rom J Morphol Embryol. 1996 Jul-Dec;42(3-4):225-34.

PMID:9168673
Abstract

A case of disseminated Langerhans cell histiocytosis (L.C.H.) in a 9 month-old boy is reported. The clinical picture was characterized by severe multivisceral involvement with cutaneous and bone lesions. Histological examination of the skin biopsy revealed diffuse infiltration of large mononucleated histiocytes admixed with a small number of inflammatory cells. Ultrastructural examination confirmed the Langerhans cell (L.C.) phenotype by showing intracytoplasmic Birbeck granules in the proliferating histiocytes. We report the histological and ultrastructural characteristics of our case and review the diagnostic features of L.C.H. in the light of the criteria established by the Writing Group of the Histiocytic Society in 1987.

摘要

报告了一例9个月大男孩的播散性朗格汉斯细胞组织细胞增多症(L.C.H.)。临床表现为严重的多脏器受累,并伴有皮肤和骨骼病变。皮肤活检的组织学检查显示,大量单核组织细胞弥漫性浸润,混有少量炎症细胞。超微结构检查通过显示增殖的组织细胞内有胞质内伯贝克颗粒,证实了朗格汉斯细胞(L.C.)表型。我们报告了该病例的组织学和超微结构特征,并根据组织细胞协会写作组1987年制定的标准,对L.C.H.的诊断特征进行了综述。

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