Tirachotikul Thitirat, Rattanathammethee Thanawat, Makruasi Nisa, Chintabanyat Anothai, Julamanee Jakrawadee, Khuhapinant Archrob, Chuncharunee Suporn, Kanitsap Nonglak, Wongkhantee Somchai, Wong Peerapon, Chaloemwong Juthatip, Praditsuktavorn Pannee, Prayongratana Kannadit, Chansung Kanchana, Phiphitaporn Pisa, Norasetthada Lalita, Intragumtornchai Tanin, Polprasert Chantana, Bunworasate Udomsak
Division of Hematology, Department of Medicine, Faculty of Medicine, Chulalongkorn University and King Chulalongkorn Memorial Hospital, Rama IV Road, Pathumwan, Bangkok, 10330, Thailand.
Division of Hematology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand.
Ann Hematol. 2024 Dec;103(12):5741-5748. doi: 10.1007/s00277-024-05953-z. Epub 2024 Aug 23.
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of T-cell lymphomas with a characteristic feature of subcutaneous nodules associated with hemophagocytic lymphohistiocytosis (HLH). Treatment options for SPTCL are mainly chemotherapy (CMT) or immunosuppressive agents with selection currently dependent on physician decisions. Outcomes between the 2 treatment remedies have not yet been comprehensively compared. This study aimed to compare complete remission (CR) rates between SPTCL patients receiving cyclosporin (CSA)-based regimen (CSA +/- steroid) and CMT. The 5-year overall survival (OS) and 5-year progression free survival (PFS) were also analyzed. Clinical data from patients with SPTCL were drawn from the Thai Lymphoma Study Group registry who were newly diagnosed between 2007 and 2023. A total of 93 patients were selected with 45 cases having received CSA-based regimen and 48 cases having received CMT. There were more patients with limited stage at skin in the CSA group (63.8% vs. 36.2%, p = 0.003), while more patients with hepato- and/or splenomegaly were found in the CMT group (56.2% vs. 24.5%; p = 0.002). Germline HAVCR2 mutations were detected in 26/33 (78.8%) cases. The CR rate was significantly higher in patients treated with CSA (87% vs. 58.3%; OR = 6.5 [95%CI, 2.7-15.3]; p = 0.002). At a median follow-up of 87.8 months (range 0-185), the 5-year OS (98% vs. 87%, p = 0.19) and PFS (72.4% vs. 69.2%, p = 0.19) showed a trend favoring patients treated with CSA. Based on our study, CSA-based regimens are the preferred first-line treatment remedy for newly diagnosed SPTCL, especially in patients with limited cutaneous involvement.
皮下脂膜炎样T细胞淋巴瘤（SPTCL）是T细胞淋巴瘤的一种罕见亚型，其特征为皮下结节伴噬血细胞性淋巴组织细胞增生症（HLH）。SPTCL的治疗选择主要是化疗（CMT）或免疫抑制剂，目前的选择取决于医生的决定。这两种治疗方法的疗效尚未得到全面比较。本研究旨在比较接受基于环孢素（CSA）方案（CSA±类固醇）和CMT的SPTCL患者的完全缓解（CR）率。还分析了5年总生存率（OS）和5年无进展生存率（PFS）。SPTCL患者的临床数据来自泰国淋巴瘤研究组登记处，这些患者于2007年至2023年期间新诊断。共选择了93例患者，其中45例接受了基于CSA的方案，48例接受了CMT。CSA组皮肤局限期患者更多（63.8%对36.2%，p = 0.003），而CMT组肝脾肿大患者更多（56.2%对24.5%；p = 0.002）。在26/33（78.8%）例中检测到种系HAVCR2突变。接受CSA治疗的患者CR率显著更高（87%对58.3%；OR = 6.5 [95%CI，2.7 - 15.3]；p = 0.002）。在中位随访87.8个月（范围0 - 185）时
