Rappersberger K, Ortel B, Forstinger C, Wolff K
Universitätsklinik für Dermatologie, Wien.
Wien Klin Wochenschr. 1994;106(10):300-8.
Cutaneous T-cell lymphomas (CTCL) show a wide clinical spectrum of cutaneous diseases caused by a clonal proliferation of malignant T-helper cells. In the early stages of CTCL diagnosis is challenging and may require a combination of clinical, pathological, immunomorphologic and molecular findings. The identification of early disease is crucial for the rapid implementation of adequate treatment, which may even be curative as has been reported with psoralen photochemotherapy (PUVA), total skin electron beam (TSEB) irradiation and topical chemotherapy. The combination of these treatment modalities with each other and, in addition, with management by synthetic retinoids and interferons has increased the therapeutic potential. Systemic (poly)-chemotherapy has been used so far exclusively for advanced stages of CTCL and may result in partial remission. Extracorporeal photochemotherapy (photopheresis) has been shown to be the most efficient mode of treatment for the Sézary syndrome.
皮肤T细胞淋巴瘤(CTCL)表现出由恶性辅助性T细胞克隆增殖引起的广泛皮肤疾病临床谱。在CTCL的早期阶段,诊断具有挑战性,可能需要综合临床、病理、免疫形态学和分子学检查结果。早期疾病的识别对于迅速实施适当治疗至关重要,正如补骨脂素光化学疗法(PUVA)、全身皮肤电子束(TSEB)照射和局部化疗所报道的那样,适当治疗甚至可能治愈疾病。这些治疗方式相互结合,此外,与合成维甲酸和干扰素联合使用,增加了治疗潜力。迄今为止,全身(多)化疗仅用于CTCL的晚期,可能导致部分缓解。体外光化学疗法(光分离置换法)已被证明是治疗塞扎里综合征最有效的治疗方式。