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Primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma (PCAE-CTL) is a rare subtype of cutaneous T-cell lymphoma with a poor prognosis. We herein report a case of PCAE-CTL accompanied by anti-Ma2 antibody-positive paraneoplastic encephalitis. A 33-year-old woman with erythema and disturbance of consciousness was diagnosed with PCAE-CTL by a skin biopsy. Head magnetic resonance imaging revealed bilateral medial temporal lobe hyperintensity, and anti-Ma2 antibody in the cerebrospinal fluid was positive. She was diagnosed with concurrent anti-Ma2 antibody-positive paraneoplastic encephalitis. In cases of lymphoma with brain lesions, invasion of the central nervous system by lymphoma and paraneoplastic encephalitis should be considered.

Background: While R-CHOP has been one of the standard therapies for untreated high-tumor-burden (HTB) follicular lymphoma (FL) for over 2 decades, obinutuzumab plus bendamustine (OB) is also currently regarded as the standard of care since its approval in 2018 in Japan; however, the long-term efficacy and safety of OB in the daily clinical practice has not been thoroughly evaluated., Methods: We conducted a multicenter retrospective study for the clinical outcome of 53 patients with HTB FL treated by OB as the frontline therapy between 2018 and 2021 in the Kyoto Hematology Clinical Study Group (KOTOSG). All patients had at least 2-year follow-up period., Results: The median age was 67, and 60.4% were classified as high risk according to the Follicular Lymphoma International Prognostic Index. The overall and complete response rates after induction therapy with OB were 98% and 83%, respectively. With a median follow-up of 38.5 months, the 3-year progression-free survival (PFS) and overall survival (OS) were 77.3% and 91.2%, respectively. Grade 3-4 hematological adverse events (AEs) were common, including neutropenia (58.5%) and lymphopenia (98.1%). Non-hematological AEs included infections, such as lung infections, coronavirus disease 2019, and sepsis, with two cases (3.8%) being fatal. Finally, propensity score-matched analysis showed no significant difference in PFS between 46 FL patients treated by the frontline OB and 46 FL patients treated by R-CHOP between 2001 and 2019 in KOTOSG., Conclusion: This study highlighted the need for careful treatment selection based on patient background and disease condition in real-world practice with more elderly patients., Competing Interests: Declarations. Conflict of interest: TT has received honoraria from Bristol Myers Squibb (BMS), Janssen Pharmaceutical, Sanofi, Kyowa Kirin, and Chugai Pharmaceutical; TK has received honoraria from BMS, Chugai Pharmaceutical, Nippon Shinyaku, Ono Pharmaceutical, Sanofi, Nippon Kayaku, AstraZeneca, Abbvie, MSD, and Janssen Pharmaceutical; EK has received honoraria from Janssen Pharmaceutical, Chugai Pharmaceutical, Astra Zeneca, Alexion Pharmaceutical, BMS, Sanofi, Kyowa Kirin, Genmab, and Meiji Seika; NU has received honoraria from BMS, Janssen Pharmaceutical, and Abbvie; SF has received honoraria from Takeda Pharmaceutical, Sanofi, Janssen Pharmaceutical, Ono Pharmaceutical, and BMS; TF has received honoraria from Takeda Pharmaceutical; SM has received honoraria from Amgen, Astellas, Otsuka Pharmaceutical, Nippon Shinyaku, Chugai Pharmaceutical, Ono Pharmaceutical, Sanofi, and BMS; YS has received honoraria from Ono Pharmaceutical, BMS, Janssen Pharmaceutical, Sanofi, Kyowa Kirin, Takeda Pharmaceutical, and Chugai Pharmaceutical. JK is a consultant for Janssen Pharmaceutical, Pfizer, Abbvie, BeiGene, and BMS; has received research funding from Kyowa Kirin, Chugai Pharmaceutical, Asahikasei, Sumitomo Pharma, Otsuka Pharmaceutical, Mochida Pharmaceutical, Taiho Pharmaceutical, and Japan Blood Products Organization; and has received honoraria from Janssen Pharmaceutical, Kyowa Kirin, Chugai Pharmaceutical, Ono Pharmaceutical, Sanofi, Pfizer, Astra Zeneca, Astellas, Eisai, Abbvie, Novartis, Daiichi Sankyo, Amgen, Otsuka Pharmaceutical, and BMS. The other authors have no conflict of interest. Ethical approval and consent to participate: The ethics committee of each participating institute approved the study. Under this approval, the need to obtain informed consent from the study subjects was waived, and we disclosed information about the survey to the patients. Patient consent for publication: Not applicable., (© 2025. The Author(s) under exclusive licence to Japan Society of Clinical Oncology.)