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蕈样肉芽肿的管理——现状与未来展望

Management of mycosis fungoides--current status and future prospects.

作者信息

Levi J A, Wiernik P H

出版信息

Medicine (Baltimore). 1975 Jan;54(1):73-88. doi: 10.1097/00005792-197501000-00004.

Abstract

Current knowledge concerning the course of mycosis fungoides and recognized prognostic factors have been reviewed. Those factors with prognostic significance at the time of biopsy diagnosis include age and the clinical findings of skin tumors, ulceration or palpable lymphadenopathy. During the course of disease, the development of skin tumors, ulceration or palpable lymphadenopathy were each associated with a poor prognosis and median survival was only 12 months if all those clinical parameters were present. Patients who developed overt visceral mycosis fungoides rarely survived more than a few months. Lymphocytopenia and the presence of malignant lymphoma in biopsied lymph nodes were also poor prognostic findings. The various modalities of therapy for proven mycosis fungoides were reviewed. Topical therapy and external irradiation were generally of symptomatic benefit only, but two recent studies have shown that aggressive use of topical nitrogen mustard and electron beam therapy are associated with long-term responses in patients with disease confined to the skin. Single agent chemotherapy often resulted in transient responses in advanced and refractory mycosis fungoides. Future approaches to the management of mycosis fungoides have been suggested. These include a thorough review of the histological features, a thorough and systematic pretreatment evaluation and randomized studies of the various treatment modalities including combination therapy in appropriately staged patients.

摘要

关于蕈样肉芽肿病程及已公认的预后因素的现有知识已被综述。活检诊断时具有预后意义的因素包括年龄以及皮肤肿瘤、溃疡或可触及淋巴结病的临床发现。在疾病过程中,皮肤肿瘤、溃疡或可触及淋巴结病的出现均与预后不良相关,如果所有这些临床参数都存在,中位生存期仅为12个月。发生明显内脏型蕈样肉芽肿的患者很少能存活超过几个月。淋巴细胞减少以及活检淋巴结中存在恶性淋巴瘤也是预后不良的表现。对已证实的蕈样肉芽肿的各种治疗方式进行了综述。局部治疗和外照射通常仅具有对症益处,但最近的两项研究表明,积极使用局部氮芥和电子束治疗与局限于皮肤的疾病患者的长期缓解相关。单药化疗在晚期和难治性蕈样肉芽肿中常导致短暂缓解。已提出了未来蕈样肉芽肿的治疗方法。这些包括对组织学特征进行全面回顾、进行全面系统的预处理评估以及对包括联合治疗在内的各种治疗方式在适当分期患者中进行随机研究。

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