title: 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.affiliation: Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, Kyoto, Japan.
authors.forename: Shinsuke
authors.initials: S
authors.lastname: Mizutani
authors.forename: Junya
authors.initials: J
authors.lastname: Kuroda
authors.forename: Yuji
authors.initials: Y
authors.lastname: Shimura
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Kobayashi
authors.forename: Yasuhiko
authors.initials: Y
authors.lastname: Tsutsumi
authors.forename: Mihoko
authors.initials: M
authors.lastname: Yamashita
authors.forename: Mio
authors.initials: M
authors.lastname: Yamamoto
authors.forename: Muneo
authors.initials: M
authors.lastname: Ohshiro
authors.forename: Nana
authors.initials: N
authors.lastname: Sasaki
authors.forename: Miki
authors.initials: M
authors.lastname: Kiyota
authors.forename: Ryuko
authors.initials: R
authors.lastname: Nakayama
authors.forename: Hitoji
authors.initials: H
authors.lastname: Uchiyama
authors.forename: Yosuke
authors.initials: Y
authors.lastname: Matsumoto
authors.forename: Shigeo
authors.initials: S
authors.lastname: Horiike
authors.forename: Shigeo
authors.initials: S
authors.lastname: Nakamura
authors.forename: Masafumi
authors.initials: M
authors.lastname: Taniwaki
mesh_terms: D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use; D016572:Cyclosporine / Q000627:therapeutic use*; D019008:Drug Resistance, Neoplasm; D005260:Female; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications*; D016399:Lymphoma, T-Cell / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy; D015434:Panniculitis / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy; D049268:Positron-Emission Tomography
chemical_list: D000970:Antineoplastic Agents; D016572:Cyclosporine