Schmuth M, Topar G, Illersperger B, Kowald E, Fritsch P O, Sepp N T
Department of Dermatology, University of Innsbruck, Innsbruck, Austria.
Cancer. 2000 Oct 1;89(7):1603-10.
Lymphomatoid papulosis is a primary cutaneous, CD30 positive lymphoproliferative disorder with the potential to transform into systemic, malignant lymphoma. Therapeutic strategies for patients with lymphomatoid papulosis have been designed to prevent transformation but have proved to be either inefficacious or limited by side effects.
The authors compared the clinical, histologic, and immunohistochemical features from a group of five patients receiving interferon-alpha (IFN-alpha) subcutaneously three times per week with the same features from a group of six patients receiving conventional therapy, including photochemotherapy, antibiotics, topical corticosteroids, or surgery, in an open trial.
In the IFN-alpha group, four patients showed a complete remission, and one patient showed a partial remission within a time period of 6 weeks. Two patients developed disease recurrences after discontinuation of short term IFN-alpha therapy (5-7 months). Thereof, one patient went into stable remission after long term IFN-alpha therapy (17 months), and one patient remains in partial remission. In the control group, one patient went into spontaneous remission, two patients showed partial remission, of which one patient developed progressive disease at a later time point, whereas three patients have recurrent disease despite of treatment.
The current results indicate that the treatment with IFN-alpha of patients with lymphomatoid papulosis alters the clinical course of the disease with fewer side effects than previous regimens; however, short term treatment does not induce stable remission. Therefore, prolonged treatment appears to be warranted for these patients.
淋巴瘤样丘疹病是一种原发性皮肤CD30阳性淋巴增殖性疾病，有转化为系统性恶性淋巴瘤的可能。针对淋巴瘤样丘疹病患者的治疗策略旨在预防转化，但已证明要么无效，要么受副作用限制。
在一项开放试验中，作者将一组每周皮下注射三次α干扰素（IFN-α）的5例患者的临床、组织学和免疫组化特征，与一组接受包括光化学疗法、抗生素、外用皮质类固醇或手术在内的传统疗法的6例患者的相同特征进行了比较。
在IFN-α组中，4例患者在6周内完全缓解，1例患者部分缓解。2例患者在短期IFN-α治疗（5 - 7个月）停药后疾病复发。其中，1例患者在长期IFN-α治疗（17个月）后进入稳定缓解期，1例患者仍处于部分缓解状态。在对照组中，1例患者自发缓解，2例患者部分缓解，其中1例患者在稍后时间点出现疾病进展，而3例患者尽管接受了治疗仍有疾病复发。
目前的结果表明，用IFN-α治疗淋巴瘤样丘疹病患者可改变疾病的临床病程，且副作用比以前的治疗方案少；然而，短期治疗不能诱导稳定缓解。因此，这些患者似乎有必要进行延长治疗。
