Hughes Charlotte Fm, Newland Kate, McCormack Christopher, Lade Stephen, Prince H Miles
Division of Haematology and Cancer Medicine, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia.
Sir Peter MacCallum Department of Oncology, University of Melbourne, Melbourne, Victoria, Australia.
Australas J Dermatol. 2016 Aug;57(3):182-91. doi: 10.1111/ajd.12349. Epub 2015 May 18.
Mycosis fungoides and Sézary syndrome are the most common variants of the cutaneous T-cell lymphomas. Assessment of a patient with a suspected diagnosis requires thorough history taking and physical examination, in combination with skin biopsy. In some cases flow cytometry, molecular studies and imaging are also required in order to diagnose and stage the disease. Staging is derived from the tumour-node-metastasis-blood classification and is currently our best attempt to stratify prognosis and hence guide management in this complex disease. Many other clinical, biological and pathological factors may help to distinguish groups at risk and predict prognosis more accurately. Management remains heavily guided by staging, such that patients with early-stage disease generally begin treatment with skin-directed or local therapies and those with advanced-stage disease have many treatment options, including chemotherapy, the use of biological agents, local and total body radiotherapy, as well as haematopoietic stem cell transplantation. Besides staging, many other patient-related factors influence the treatment strategy, particularly where symptom relief is paramount. There are many challenges remaining in the study of Mycosis fungoides and Sézary syndrome and, given the rarity of the disease, concerted worldwide efforts are required to conduct efficient and effective research.
蕈样肉芽肿和 Sézary 综合征是皮肤 T 细胞淋巴瘤最常见的亚型。对疑似诊断的患者进行评估需要全面的病史采集和体格检查，并结合皮肤活检。在某些情况下，还需要流式细胞术、分子研究和影像学检查来诊断疾病并进行分期。分期源自肿瘤-淋巴结-转移-血液分类，目前是我们对这种复杂疾病的预后进行分层并因此指导治疗的最佳尝试。许多其他临床、生物学和病理学因素可能有助于区分高危人群并更准确地预测预后。治疗仍主要由分期指导，因此早期疾病患者通常开始采用针对皮肤或局部的治疗方法，而晚期疾病患者有多种治疗选择，包括化疗、使用生物制剂、局部和全身放疗以及造血干细胞移植。除了分期，许多其他与患者相关的因素也会影响治疗策略，尤其是在缓解症状至关重要的情况下。在蕈样肉芽肿和 Sézary 综合征的研究中仍存在许多挑战，鉴于该疾病的罕见性，需要全球共同努力开展高效且有效的研究。
