朗格汉斯细胞组织细胞增多症:具有相同组织病理基础的多种表现。

Anales Espanoles De Pediatria Pub Date : 2002-11-01
J Martínez Baylach, N Pardo García, M Torrent Español, E Moliner Calderón, I Anquela Sanz, J Cubells Rieró
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引用次数: 0

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,是一种罕见的疾病。其特点是组织细胞、嗜酸性粒细胞和朗格汉斯细胞聚集和增殖,电镜下可见Birbeck颗粒。它涉及单个器官或系统,也可以表现为多系统疾病。临床表现可能有很大差异,从自发消退的良性自限性到缓慢进展的恶性疾病。我们报告了5例LCH病例,其组织病理基础相同,但结果不同。
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[Langerhans' cell histiocytosis: various manifestations with the same histopathologic base].

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.

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