Roupe G, Sandström M H, Kjellström C
Department of Dermatology, Sahlgrenska University Hospital, Göteborg, Sweden.
Acta Derm Venereol. 1996 Nov;76(6):475-8. doi: 10.2340/0001555576475478.
We report a follow-up for 3-18 years of 24 patients with the non-infiltrated patch and infiltrated plaque stage of mycosis fungoides, treated with psoralen photochemotherapy (PUVA) and on resistant infiltration or development of tumours also with fractionated radiation therapy. All patients with patch and limited plaques showed complete remission after initial PUVA therapy. Half of the patients with patch stage mycosis fungoides and also half of the patients with plaque stage mycosis fungoides were in complete remission when the study ended. Most of them had remission periods for years after early PUVA treatment. Patients with more advanced mycosis fungoides needed repeated periods of PUVA therapy. Two patients with extensive infiltrated plaques did not reach complete remission at all during the study but progressed and finally died of their T-cell lymphoma. Another 4 patients with extensive plaque stage mycosis fungoides died after initial complete remission for a maximum of 3 years. In this investigation the clinical evaluation was made by one dermatologist and the histopathological evaluation by one pathologist. This is of importance since in the early stages of mycosis fungoides the diagnosis is challenging and may require a combination of clinical, histopathological and molecular evaluations. The identification of early disease is crucial for the rapid implementation of adequate treatment. The study shows that early PUVA therapy may delay extracutaneous spread and possibly also in some cases be curative.
我们报告了24例蕈样肉芽肿非浸润性斑块期和浸润性斑块期患者的3至18年随访情况,这些患者接受了补骨脂素光化学疗法(PUVA)治疗,对于耐药性浸润或肿瘤形成还采用了分次放射治疗。所有斑块期和局限性斑块期患者在初始PUVA治疗后均显示完全缓解。研究结束时,斑块期蕈样肉芽肿患者中有一半以及斑块期蕈样肉芽肿患者中也有一半处于完全缓解状态。他们中的大多数在早期接受PUVA治疗后有多年的缓解期。病情更严重的蕈样肉芽肿患者需要反复进行PUVA治疗。两名广泛性浸润性斑块患者在研究期间根本未达到完全缓解,病情进展,最终死于T细胞淋巴瘤。另外4例广泛性斑块期蕈样肉芽肿患者在初始完全缓解最多3年后死亡。在这项研究中,临床评估由一名皮肤科医生进行,组织病理学评估由一名病理学家进行。这一点很重要,因为在蕈样肉芽肿的早期阶段,诊断具有挑战性,可能需要结合临床、组织病理学和分子评估。早期疾病的识别对于迅速实施适当治疗至关重要。该研究表明,早期PUVA治疗可能会延迟皮肤外扩散,在某些情况下可能也具有治愈作用。