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[朗格汉斯细胞组织细胞增多症:具有相同组织病理学基础的多种表现]

[Langerhans' cell histiocytosis: various manifestations with the same histopathologic base].

作者信息

Martínez Baylach J, Pardo García N, Torrent Español M, Moliner Calderón E, Anquela Sanz I, Cubells Rieró J

机构信息

Servicio de Pediatría. Universitat Autònoma de Barcelona. Hospital de la Santa Creu i Sant Pau. Barcelona. España.

出版信息

An Esp Pediatr. 2002 Nov;57(5):484-7.

Abstract

Langerhans' cell histiocytosis (LCH), previously known as histiocytosis X, is a rare disease. It is characterized by the accumulation and proliferation of histiocytes, eosinophils and Langerhans' cells with Birbeck granules detected by electron microscopy. It involves single organs or systems or can present as a multisystem disease. The clinical presentation may vary widely, ranging from benign self-limiting types with spontaneous regression to slowly-progressive malignant disease. We report five cases of LCH with the same histopathologic basis but different outcome.

摘要

朗格汉斯细胞组织细胞增多症(LCH),以前称为组织细胞增多症X,是一种罕见疾病。其特征是组织细胞、嗜酸性粒细胞和朗格汉斯细胞的积聚与增殖,通过电子显微镜可检测到具有Birbeck颗粒。它可累及单个器官或系统,也可表现为多系统疾病。临床表现差异很大,从具有自发消退的良性自限型到缓慢进展的恶性疾病。我们报告了5例具有相同组织病理学基础但结局不同的LCH病例。

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