Successful Treatment of Disseminated Subcutaneous Panniculitis-Like T-Cell Lymphoma with Single Agent Oral Cyclosporine as a First Line Therapy

Abstract

Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a rare cutaneous neoplasm of mature cytotoxic T-cells. Currently there are no standardized therapies for SPTL; however good responses have been seen with chemotherapy regimens generally employed for B-cell lymphomas. Cyclosporine, an immunosuppressant, has shown good responses in relapsed/refractory SPTL; however its use in first line setting is not well established. We, herein, describe a 22-year-old girl with disseminated SPTL who attained complete clinical remission with single agent oral cyclosporine used as a first line therapy.

Subcutaneous panniculitis-like T-cell lymphoma with macrophage activation syndrome treated by cyclosporine and prednisolone

“…Several refractory as well as naïve cases of SPTCL (with or without HPS) have been successfully treated and kept in long-term remissions by cyclosporine alone or with other immunosuppressive agents. [9101112] We combined high-dose oral steroids with cyclosporine to control the severe systemic symptoms (persisting high-grade fever and HPS).…”

Subcutaneous panniculitis-like T-cell lymphoma: The authors’ experience of diagnosis and treatment

“…В ряде случаев применялась также высокодозная ПХТ с трансплантацией аутологичных или аллогенных гемопоэтических стволовых клеток крови. Однако в настоящее время появились работы, авторы которых приходят к выводу, что при ППТКЛ без гемофагоцитарного синдрома проведение ПХТ не оправдано и что монотерапия преднизолоном или другими иммуносупрессивными препаратами дает сходные результаты лечения [5,23,24]. Более того, в настоящее время ППТКЛ рассматривается некоторыми автора-таблица 2.…”

Subcutaneous Panniculitis-like T-cell Lymphoma: Review of Therapies

“…Fludarabine-based chemotherapies have shown overall remission rates of more than 70% in a few case reports [35,36]. Doxorubicin-based therapies have achieved complete or partial complete remission rates of 50% [37]. Because the presence of HPS at diagnosis is one of the most important prognostic factors that predict poor overall survival, it may be a useful parameter to determine if disease is indolent or aggressive [2,10].…”

“…Several reports suggest that SPTCL patients without HPS may benefit from cyclosporine and steroids [2,44]. Upfront use of cyclosporine was able to maintain durable remission in patient with disseminated SPTCL [37]. A 22-year-old girl with disseminated SPTCL attained complete clinical remission with single agent oral cyclosporine used as a first line therapy.…”