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蕈样肉芽肿和塞扎里综合征的诊断

Diagnostics in mycosis fungoides and Sezary syndrome.

作者信息

Olek-Hrab Karolina, Silny Wojciech

机构信息

Department of Dermatology of Karol Marcinkowski University of Medical Sciences in Poznań, Poland.

出版信息

Rep Pract Oncol Radiother. 2013 Dec 30;19(2):72-6. doi: 10.1016/j.rpor.2013.11.001. eCollection 2014 Mar.

Abstract

AIM

The aim of this paper was to present diagnostic methods helping in the recognition of mycosis fungoides (MF) and Sezary syndrome (SS).

BACKGROUND

Mycosis fungoides is the most common form of primary cutaneous T-cell lymphomas. It is characterized by a distinctive long-term course and malignant T-cell proliferation. MF diagnosis is not easy, mainly due to the atypical clinical presentation of the disease at an early stage.

MATERIALS AND METHODS

Low specific changes, which can be observed at the histopathological examination. Initially, the skin lesions may resemble psoriasis, atopic dermatitis or chronic eczema. Patients are qualified according to the available, and generally accepted WHO-EORTC classification, based on a combination of clinical and histopathological markers. From a clinical point of view, it is also important to carry out the qualification according to the TNMB assessment, which allows to specify the stage of the disease, and is helpful in the monitoring of the course of disease and therapeutic effects.

RESULTS

In this paper we try to present currently available diagnostic methods.

CONCLUSION

Diagnosis of MF and SS still causes many problems due to less characteristic changes in the early stage of disease and requires wide interdisciplinary knowledge.

摘要

目的

本文旨在介绍有助于诊断蕈样肉芽肿(MF)和 Sézary 综合征(SS)的方法。

背景

蕈样肉芽肿是原发性皮肤 T 细胞淋巴瘤最常见的形式。其特点是病程独特且存在恶性 T 细胞增殖。MF 的诊断并不容易,主要是因为该疾病在早期临床表现不典型。

材料与方法

在组织病理学检查中可观察到的低特异性变化。最初,皮肤病变可能类似银屑病、特应性皮炎或慢性湿疹。根据现有的、普遍接受的 WHO-EORTC 分类标准,结合临床和组织病理学指标对患者进行诊断。从临床角度来看,根据 TNMB 评估进行诊断也很重要,这有助于明确疾病分期,并有助于监测疾病进程和治疗效果。

结果

在本文中,我们试图介绍目前可用的诊断方法。

结论

由于疾病早期特征性变化较少,MF 和 SS 的诊断仍然存在许多问题,需要广泛的多学科知识。

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本文引用的文献

5
Mycosis fungoides: a dermatological masquerader.蕈样肉芽肿:一种皮肤伪装者。
Br J Dermatol. 2007 Jan;156(1):1-10. doi: 10.1111/j.1365-2133.2006.07526.x.
6
WHO-EORTC classification for cutaneous lymphomas.世界卫生组织-欧洲癌症研究与治疗组织皮肤淋巴瘤分类
Blood. 2005 May 15;105(10):3768-85. doi: 10.1182/blood-2004-09-3502. Epub 2005 Feb 3.
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Granulomatous slack skin.肉芽肿性皮肤松弛症
Dermatol Clin. 1994 Apr;12(2):375-89.
10
Sézary syndrome: diagnosis, prognosis, and critical review of treatment options.
J Am Acad Dermatol. 1990 Mar;22(3):381-401. doi: 10.1016/0190-9622(90)70054-l.

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