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蕈样肉芽肿

[Mycosis fungoides].

作者信息

Graciansky P D, Guilaine J

出版信息

Ann Med Interne (Paris). 1975 Mar;126(3):161-9.

PMID:123718
Abstract

The classical characteristics of Myocsis Fungoides are discussed. 1) The clinical characteristics of the three forms: tumour from the start, erythrodermic and polymorphic forms of Alibert-Buzin. 2) Histological characteristics : dense dermal infiltrates or epidermotropic infiltration, or around a more or less malignant histiocytic reaction, there develops a host reaction, resembling a granuloma. During the course of the disease, one may observe lymphocyte depletion, whilst delayed hypersensitivity tests become negative. The various types of lymphadenopathy are analysed, the possibility of visceral lesions confirmed. However, knowledge of Sezary's syndrome makes his concept of the disease doubtful. A pathological picture similar to Mycosis Fungoides may be observed during Sezary's syndrome, with circulating giant cells. The presence of small Sezary cells, Lutzner-Flandrin variety, is common in the dermal infiltrate, and even more common in the lymph node visceral infiltrates of Mycosis Fungoides. Thus, one is led to the concept according to which the primary phenomenon is probably pathological proliferation of abnormal T lymphocytes (large and small Sezary cells), the histiocytic proliferation would then simply be a secondary reaction. This theory is then discussed.

摘要

本文讨论了蕈样肉芽肿的典型特征。1) 三种形式的临床特征:一开始即为肿瘤型、红皮病型和阿利贝尔 - 比赞多形型。2) 组织学特征:致密的真皮浸润或亲表皮浸润,或在或多或少的恶性组织细胞反应周围,会出现类似肉芽肿的宿主反应。在疾病过程中,可能会观察到淋巴细胞减少,同时迟发型超敏反应试验呈阴性。分析了各种类型的淋巴结病,证实了内脏病变的可能性。然而,对塞扎里综合征的了解使他对该疾病的概念产生了怀疑。在塞扎里综合征期间,可能会观察到与蕈样肉芽肿相似的病理图像,伴有循环巨细胞。小塞扎里细胞(卢茨纳 - 弗兰德林变种)的存在在真皮浸润中很常见,在蕈样肉芽肿的淋巴结内脏浸润中更为常见。因此,人们得出这样的概念,即主要现象可能是异常T淋巴细胞(大、小塞扎里细胞)的病理性增殖,组织细胞增殖则可能只是一种继发反应。然后对这一理论进行了讨论。

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