Abstract

Primary cutaneous CD30+ lymphoproliferative disorders (CD30+ LPDs) includes a spectrum of disorders such as lymphomatoid papulosis (LyP), borderline cases of CD30+ LPDs, and primary cutaneous anaplastic large cell lymphoma (PCALCL). A broad variety of therapeutic strategies for these diseases have been reported and range from simple observation to aggressive systemic chemotherapy. In this respect, this study was conducted to investigate the effectiveness and outcome of methotrexate (MTX) treatment in primary cutaneous CD30+ LPDs. Patient charts including response to MTX and long-term follow-up data and clinical and histopathologic data of 25 patients of LyP and 6 patients of PCALCL were retrospectively assessed. he patients ages ranged from 5 to 65 years. Mean initial dose of MTX was 14.8 mg/week. Mean duration of MTX therapy per each cycle was 10 weeks in LyP patients and 13.7 weeks in PCALCL patients. Among 25 patients of LyP, 19 patients(76%) showed complete remission (CR), 5 patients(20%) showed partial remission (PR) and 1 patient(4%) showed no response (NR). Among 6 patients of PCALCL, 5 patients(83.3%) showed CR and 1 patient(16.7%) showed PR. During mean follow-up of 87 months, 6 patients (24%) of LyP showed relapse. During mean follow-up of 94.8 months, 5 (83.3%) of PCALCL showed relapse. PCALCL developed in 1 patient of LyP. Mycosis fungoides developed in 2 patients of PCALCL and LyP developed in 3 of PCALCL. At the final follow up, 2 patients of PCALCL survived more than 10 years and none of LyP and PCALCL patients died. Based on this study, we suggest that low dose MTX could be highly beneficial not only for LyP and multifocal PCALCL but also for solitary or localized PCALCL. Low-dose MTX is an effective and safe treatment for primary cutaneous CD30+ LPDs.

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