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

Abstract

Summary Mycosis fungoides (MF) is a primary cutaneous T‐cell lymphoma with unfavourable prognosis for patients with 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 MF, a subtype of MF with poorer prognosis, in a 59‐year‐old woman. She was initially diagnosed with MF restricted to the skin, of T3N0M0B0/stage IIB according to the current World Health Organization–European Organisation for Research and Treatment of Cancer 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 cytarabine chemotherapy, followed by high‐dose chemotherapy with thiotepa and carmustine with autologous SCT. Despite rapid response and complete remission of the cerebral lesions, disease recurrence of the skin occurred soon after. Interestingly, readministration 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 17 months after autologous SCT. What's already known about this topic? Mycosis fungoides is a primary cutaneous T‐cell lymphoma with unfavourable prognosis for the advanced stages of the disease. A refractory course of disease requires systemic therapy. What does this study add? We report on an unusual case of a patient with mycosis fungoides with cerebral involvement, in which a durable complete remission was achieved upon autologous stem cell therapy and interferon alfa‐2a maintenance therapy.

mRNA-Based Anti-TCR CDR3 Tumour Vaccine for T-Cell Lymphoma

“…Of note, although injections of mice are performed with 20 micrograms of RNA per injection, this formulation was previously demonstrated by Kranz et al to be efficacious at a similar dose (7.2 to 29 micrograms) per injection in humans [ 12 ]. This type of vaccination can be advantageously combined with immune checkpoint inhibitors currently under evaluation in T-lymphomas [ 37 , 38 , 39 , 40 ] or established immunomodulatory approaches, especially interferon-alpha [ 41 , 42 ]. The possibility of vaccinating against the TCR to treat CTCL is further supported by other reports: Berger et al [ 43 ], Zheng et al [ 44 ] and Winter et al [ 45 ] have shown that MHC class I epitopes derived from TCR chains expressed in human CTCL can be recognised by cytotoxic T-cells.…”

Mycosis Fungoides and Sézary Syndrome

“…1 A case study of a patient with mycosis fungoides showed that the use of alpha interferon after autologous stem cell transplantation maintained sustained complete remission of at least 17 months. 55 The retinoid bexarotene, which is used as a topical agent in the earlier stages of the disease, may also be used as a systemic treatment, e.g. in combination with PUVA.…”

Pathogenesis and Therapy of Primary Cutaneous T-Cell Lymphoma: Collegium Internationale Allergologicum (CIA) Update 2020

“…If and how exactly these mechanisms contribute to treatment resistance in SS cell subpopulations has yet to be addressed in detail. Taken together, despite the numerous systemic treatment options available, long – term remission rates with conventional treatments alone are still low and allogeneic hematopoietic stem cell transplantation (alloHSCT) is currently the only curative option in advanced MF/SS [85, 86].…”