Interferon alfa‐2a maintenance after salvage autologous stem cell transplantation in atypical mycosis fungoides with central nervous system involvement

Abstract

Mycosis fungoides is a primary cutaneous T‐cell lymphoma with unfavorable prognosis for the advanced stages of the disease. Refractory disease and advanced‐stage disease require systemic therapy.

We report on a rare case of an atypical predominantly CD8+ folliculotropic mycosis fungoides (MF), a subtype of MF with poorer prognosis, in a 59‐year‐old woman, initially diagnosed with MF restricted to the skin‐ of T3N0M0B0/stage IIB according to the current WHO/EORTC classification. First‐line treatment with local percutaneous radiotherapy in combination with systemic interferon alfa‐2a resulted in complete remission. However, 21 months later the disease progressed to T3N0M1B0/stage IVB with development of cerebral manifestation and thus very poor prognosis. Allogeneic stem cell transplantation (SCT) was not a therapeutic option due to the lack of a suitable donor. We initiated methotrexate and cytarabin chemotherapy, followed by high‐dose chemotherapy with thiotepa and BCNU with autologous SCT. Despite rapid response and complete remission of the cerebral lesions, disease recurrence of the skin occurred soon after. Interestingly, re‐administration of interferon alfa‐2a as a maintenance treatment after the salvage autologous SCT resulted in a durable complete remission during the follow‐up period of currently 13 months after autologous SCT.

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