Cyclosporine A for chemotherapy-resistant subcutaneous panniculitis-like T cell lymphoma with hemophagocytic syndrome.

Abstract

Subcutaneous panniculitis-like T cell lymphoma (SPTL) is a rare subtype of non-Hodgkin lymphoma for which a definitive therapeutic strategy has not been established yet. We report a case of chemotherapy-resistant SPTL with hemophagocytic syndrome (HPS) which was successfully treated with cyclosporine A (CsA) plus methylprednisolone (mPSL), and also reviewed 11 SPTL cases treated with CsA, previously reported in the literature. Our patient was a 38-year-old female with SPTL. The disease progressed despite conventional chemotherapy using cytotoxic agents including alkylators, anthracyclins or purine analogues, and, after 2 months of chemotherapy, was eventually complicated by HPS and disseminated intravascular coagulation (DIC). CsA (4 mg/kg/day) plus mPSL treatment dramatically improved HPS with DIC, reduced subcutaneous tumors within 2 weeks, and finally induced complete remission (CR) after 3 months. Currently, the patient has maintained CR while being treated with CsA for 12 months. In addition to our case, 9 of 11 SPTL cases were successfully treated with CsA, and 8 were induced to CR. Time to first response to CsA was within 2 weeks in most cases, regardless of prior treatment or the co-occurrence of HPS. Our case and this first comprehensive review on CsA for SPTL suggest that CsA may constitute a candidate treatment strategy for SPTL.

• Authors: Shinsuke Mizutani, Junya Kuroda, Yuji Shimura, Tsutomu Kobayashi, Yasuhiko Tsutsumi, Mihoko Yamashita, Mio Yamamoto, Muneo Ohshiro, Nana Sasaki, Miki Kiyota, Ryuko Nakayama, Hitoji Uchiyama, Yosuke Matsumoto, Shigeo Horiike, Shigeo Nakamura, Masafumi Taniwaki
• Journal: Acta haematologica (Acta Haematol) Vol. 126 Issue 1 Pg. 8-12 ( 2011) ISSN: 1421-9662 [Electronic] Switzerland
• PMID: 21411984 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2011 S. Karger AG, Basel.

Chemical References

• Antineoplastic Agents
• Cyclosporine

Topics

• Adult
• Antineoplastic Agents (therapeutic use)
• Cyclosporine (therapeutic use)
• Drug Resistance, Neoplasm
• Female
• Humans
• Lymphohistiocytosis, Hemophagocytic (complications)
• Lymphoma, T-Cell (complications, diagnostic imaging, drug therapy)
• Panniculitis (complications, diagnostic imaging, drug therapy)
• Positron-Emission Tomography