Low-dose Methotrexate Treatment for Solitary or Localized Primary Cutaneous Anaplastic Large Cell Lymphoma: A Long-term Follow-up Study

Abstract

Primary cutaneous CD30 + lymphoproliferative disorders are the second most common cutaneous T-cell lymphoma. In the case of solitary or localized primary cutaneous anaplastic large cell lymphoma (PCALCL), surgical excision or radiotherapy are the first-line treatments. However, those options can lead to significant functional or cosmetic morbidities. Low-dose methotrexate has not been established in treatment for solitary or localized PCALCL. Furthermore, even in multifocal PCALCL, low-dose methotrexate has been proposed as first-line therapy based on only a limited number of studies. There has been no report of long-term follow-up data focused specifically on Asian patients with PCALCL treated with low-dose methotrexate. This study suggests that low-dose MTX treatment should be established as an effective alternative in the treatment guidelines for solitary or localized PCALCL, in which surgical excision and/or radiotherapy is not feasible. Although low-dose methotrexate (MTX) has been used widely in treatment of a variety of dermatological diseases, including multifocal primary cutaneous anaplastic large cell lymphoma (PCALCL), it has not been established for use in the treatment guidelines for solitary or localized PCALCL. Furthermore, there has been no report of long-term follow-up data in Asian patients with PCALCL treated with low-dose MTX. To investigate the effectiveness and clinical outcome of treatment with low-dose MTX, clinical and longterm follow-up data of 7 patients with solitary or localized PCALCL were analysed retrospectively. Of the 7 patients, 6 (85.7%) showed a complete response and 1 (14.3%) showed partial remission. During follow-up, mean duration of 92.1 months, 5 patients developed one or more cutaneous relapses. At the last follow-up, all of the patients with PCALCL were alive without disease. These results indicate that low-dose MTX is a highly effective and safe treatment for solitary or localized PCALCL as well as multiple relapsed lesions.

A patient with multiple nodules and plaques on forehead, cheeks and chin

“…MTX has been used for a variety of dermatological disorders presumably for its anti‐inflammatory and antiproliferative effects 8 . Low‐dose MTX is well‐tolerated and has few side‐effects even when prescribed for long‐term maintenance treatment over months and years 9 …”

“…8 Lowdose MTX is well-tolerated and has few side-effects even when prescribed for long-term maintenance treatment over months and years. 9 Despite PCMZL being an indolent lymphoma with an excellent response to treatment, cutaneous recurrences can occur. In patients with indolent behaviour type of lymphoma such as PCMZL, follow-up intervals should be done every 6 months as recommended by Chen et al in their study.…”

Primary Cutaneous Anaplastic Large Cell Lymphoma Arising in a Patient with Rhupus Syndrome and Sjogren’s Syndrome

“… 1 , 11 And evidences about the efficacy of low-dose MTX in the treatment of PC-ALCL are increasing. 12–14 Park et al 14 assessed the long-term follow-up data of 7 patients with solitary or localized PC-ALCL and of them, 6 (85.7%) showed a complete response and 1 (14.3%) showed partial remission. Therefore in our case, MTX was continued and the patient was treated with radiotherapy.…”