-Author name in bold denotes the presenting author
-Asterisk * with author name denotes a Non-ASH member
Clinically Relevant Abstract denotes an abstract that is clinically relevant.

PhD Trainee denotes that this is a recommended PHD Trainee Session.

Ticketed Session denotes that this is a ticketed session.

3059 Treatment and Outcome of ATL Diagnosed in 2021 to 2023 By the Kagoshima ATL Registry

Program: Oral and Poster Abstracts
Session: 625. T Cell, NK Cell, or NK/T Cell Lymphomas: Clinical and Epidemiological: Poster II
Hematology Disease Topics & Pathways:
Clinical Practice (Health Services and Quality)
Sunday, December 8, 2024, 6:00 PM-8:00 PM

Daisuke Nakamura, MD, PhD1,2*, Satsuki Owatari, MD, PhD3*, Takayoshi Miyazono, MD, PhD4*, Hidetoshi Nakashima, MD5*, Akihiko Arai, MD, PhD6*, Hirosaka Inoue, MD7*, Kentaro Yonekura, MD, PhD8*, Kazuyuku Fujii, MD, PhD9*, Shogo Takeuchi, MD10*, Satoshi Fukutoku, MD1,5*, Seiichiro Nakabeppu, MD11*, Kodai Shima, MD1,12*, Naosuke Arima, MD1,13*, Yuhei Kamada, MD, PhD1,3*, Koichi Haraguchi, MD, PhD3*, Hideaki Kawada, MD, PhD6*, Yoshikiyo Ito, MD, PhD4*, Maki Otsuka, MD, PhD3*, Shuichi Hanada, MD, PhD14, Makoto Yoshimitsu, MD, PhD15 and Kenji Ishitsuka, MD, PhD16

1Department of Hematology and Rheumatology, Kagoshima University Hospital, Kagoshima, Japan
2Department of Hematology, Saiseikai Sendai Hospital, Kagoshima, Japan
3Department of Hematology, Kagoshima Medical Center, Kagoshima, Japan
4Department of Hematology, Imamura General Hospital, Kagoshima, Japan
5Department of Hematology, Seijinkai Ikeda Hospital, Kagoshima, Japan
6Department of Hematology and Rheumatology, Kagoshima City Hospital, Kagoshima, Japan
7Department of Hematology, Imakire General Hospital, Kagoshima, Japan
8Department of Dermatology, Imamura General Hospital, Kagoshima, Japan
9Department of Dermatology, Kagoshima University Hospital, Kagoshima, Japan
10Department of Hematology, Izuro Imamura Hospital, Kagoshima, Japan
11Department of Hematology, Izumi General Medical Center, Kagoshima, Japan
12Department of Hematology, Kirishima Medical Center, Kagoshima, Japan
13Department of Hematology, Izumi Regional Medical Center, Kagoshima, Japan
14Department of General Internal Medicine, Ibusuki Medical Center, Kagoshima, Japan
15Department of Hematology and Rheumatology, Graduate School of Medical and Dental Sciences, Kagoshima University Hospital, Kagoshima, Japan
16Kagoshima University Hospital, Kagoshima, Japan

Introduction: Adult T-cell leukemia/lymphoma (ATL) is a peripheral T-cell malignancy with very poor prognosis. We have initiated a all-case registration of ATL patients (Kagoshima ATL registry) to reveal the epidemiology, clinical profile and prognosis of ATL by constructing a database of ATL patients diagnosed in Kagoshima, Japan, one of the most pandemic areas for ATL in the world.

Methods: We prospectively collected clinical information in newly diagnosed patients with ATL on or after April 1, 2021 at all facilities in Kagoshima Prefecture providing hematology services. The follow-up data in registered patients was then collected annually. This study was approved by the ethics committee of each institution.

Results and Discussion; A total of 167 patients were registered during the period of April 1, 2021 to March 31, 2023, of which 161 patients were included in this analysis by excluding 3 patients who were not met the criteria of registration and 3 patients who had duplicated registration. The number of patients was 83, 52, 15, and 13 in acute, lymphoma, chronic, and smoldering types, respectively. In 161 cases, aggressive ATL (acute- and lymphoma-type, and chronic-type with unfavorable prognostic factors) and indolent ATL (smoldering-type and chronic-type without any unfavorable prognostic factors) was 143 cases, 18 cases, respectively. The median age of onset was 72 years old. The median observation period was 19.8 (0.6-35) months, and the median survival was 6.8, 11.6 months, not-reached, and not-reached for acute, lymphoma, chronic, and smoldering types, respectively. Survival stratification by the simplified ATL-prognostic index (sATL-PI) at diagnosis and the Japan Clinical Oncology Group (JCOG)-PI for acute and lymphoma-type ATL, and aggressive ATL were confirmed, respectively (P<0.0001). In aggressive ATL, 83.9 % (120/143) of patients received first-line treatment. Among them, CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone), VCAP-AMP-VECP (VCAP: vincristine, cyclophosphamide, doxorubicin, and prednisolone, AMP: doxorubicin, ranimustine, and prednisolone, and VECP: vindesine, etoposide, carboplatin, and prednisolone), any chemotherapy by cytotoxic agents combined with mogamulizumab (Moga+CTx), and CHP therapy combined with brentuximab vedotin (BV-CHP) were performed in 30.0 %, 28.3 %, 28.3 %, and 13.4% of patients, respectively. Allogeneic stem cell transplantation (Allo-HSCT) was performed in 11.2% (16/143) patients with aggressive ATL with a median age of 62.5 years (44-72 years). Allo-HSCT was performed in 25.9 % (15/58) of patients who were equal or younger than 70 years old at diagnosis. In 34 patients who received Moga+CTx and 16 patients who received BV-CHP in the first-line therapy, 94.1% and 68.7% of patients acquired CR or PR, respectively (P=0.073). The time to next treatment in patients who received Moga+CTx and BV-CHP were 8.1 and 3.2 months, respectively (P=0.0231), indicating that the remission by Moga+CTx was more durable than the BV-CHP in the first-line treatment. Second-line treatment was applied to 81 patients. BV or Moga with or without CTx were most frequently used in the second-line treatment (n=81). This registration is expected to provide real-world data of ATL by regional registry in one of the worlds most endemic area in HTLV-1 under the circumstances of the decreasing of HTLV-1 infected individuals in younger people and recent introduction of novel therapeutic agents including tucidinostat and valemetostat in Japan.

Disclosures: Nakamura: Janssen Pharmaceutical K.K.: Honoraria; Kyowa Kirin Co.,Ltd: Honoraria; Takeda Pharmaceutical Co., Ltd.: Honoraria; Meiji Seika: Honoraria; Chugai Pharmaceutical Co., Ltd.: Honoraria; PharmaEssentia Corp.: Honoraria; Bristol-Myers Squibb K.K.: Honoraria; Daiichi Sankyo Co., Ltd.: Honoraria; AbbVie GK: Honoraria; ASAHI KASEI PHARMA CORPORATION: Honoraria. Owatari: Takeda Pharmaceutical Co., Ltd.: Honoraria; Meiji Seika: Honoraria; Genmab: Honoraria; AbbVie: Honoraria; Janssen Pharmaceutical K.K.: Honoraria; Daiichi Sankyo Co., Ltd.: Honoraria. Miyazono: AbbVie: Honoraria; Janssen: Honoraria; Chugai Pharmaceutical Co., Ltd.: Honoraria; Meiji Seika: Honoraria; Sanofi: Honoraria; Nippon Shinyaku Co., Ltd.: Honoraria; Novartis Pharma K.K.: Honoraria; JCR Pharmaceuticals Co., Ltd.: Honoraria; JIMRO Co., Ltd.: Honoraria; ASAHI KASEI PHARMA CORPORATION: Honoraria. Nakashima: Chugai Pharmaceutical Co., Ltd.: Honoraria; Takeda Pharmaceutical Co., Ltd.: Honoraria; Meiji Seika: Honoraria; Sanofi: Honoraria; Janssen: Honoraria; ONO PHARMACEUTICAL CO., LTD.: Honoraria; Nippon Kayaku Co., Ltd.: Honoraria. Arai: PharmaEssentia Corp.: Honoraria. Yonekura: Meiji Seika: Honoraria; TAIHO PHARMA: Honoraria; Eisai Co., Ltd.: Honoraria; Kyowa Kirin Co., Ltd.: Honoraria; UCBCares Japan.: Honoraria; Nippon Boehringer Ingelheim Co., Ltd.: Honoraria; Eli Lilly Japan K.K.: Honoraria; Sanofi: Honoraria; Daiichi Sankyo Co., Ltd.: Honoraria; LEO Pharma: Honoraria; Janssen Pharmaceutical K.K.: Honoraria; Bristol-Myers Squibb K.K.: Honoraria; Maruho Machinery Solutions Inc.: Honoraria; Minophagen: Honoraria; Sun Pharmaceutical Industries Limited: Honoraria; Eisai Co., Ltd.: Honoraria; AbbVie: Honoraria; SATO PHARMACEUTICAL CO., LTD.: Honoraria. Takeuchi: Sanofi: Honoraria. Fukutoku: Meiji Seika: Honoraria. Shima: AbbVie: Honoraria. Kamada: Janssen: Honoraria; Sanofi: Honoraria; Chugai Pharmaceutical Co., Ltd.: Honoraria; Takeda Pharmaceutical Co., Ltd.: Honoraria; ONO PHARMACEUTICAL CO., LTD.: Honoraria; AbbVie: Honoraria; Nippon Shinyaku Co., Ltd.: Honoraria. Kawada: Meiji: Honoraria. Ito: Chugai Pharmaceutical Co., Ltd.: Honoraria; Bristol-Myers Squibb K.K.: Honoraria; Astellas Pharma Inc.: Honoraria; AstraZeneca K.K.: Honoraria; MSD: Honoraria; AbbVie: Honoraria; AMGEN: Honoraria; Meiji Seika Pharma Co., Ltd.: Honoraria; Otsuka Pharmaceutical Co., Ltd.: Honoraria; Novartis: Honoraria. Hanada: Kyowa Kirin Co., Ltd.: Honoraria. Yoshimitsu: Takeda: Honoraria; Ono Pharmaceutical: Honoraria; Bristol-Myers Squibb Company: Honoraria; Kissei: Honoraria; Daiichi Sankyo: Honoraria; Genmab: Honoraria; Sanofi: Honoraria; PharmaEssentia Corp.: Honoraria; Meiji Seika Pharma Co., Ltd: Honoraria; Asteras: Honoraria; Novartis: Honoraria; Chugai Pharmaceutical: Honoraria; Nippon Kayaku Co., Ltd.: Honoraria. Ishitsuka: Kyowa Kirin Co., Ltd.: Consultancy, Honoraria, Other: All support for the present manuscript (e.g., funding, provision of study materials, medical writing, article processing charges, etc.) ; Ono Pharmaceutical Co., Ltd.: Honoraria, Other: Grants or contracts from any entity.; Kyowa Kirin Co., Ltd.: Consultancy, Honoraria, Other: Grants or contracts from any entity.; Abbvie: Honoraria, Other: Grants or contracts from any entity.; Genmab: Honoraria, Other: Grants or contracts from any entity.; Daiichi Sankyo: Consultancy, Honoraria, Other: Grants or contracts from any entity.; Pfizer: Other: Grants or contracts from any entity.; Bristol Myers Squibb: Honoraria, Other: Grants or contracts from any entity.; Mitsubishi Tanabe pharma: Other: Grants or contracts from any entity.; Incyte: Other: Grants or contracts from any entity.; Yakult: Consultancy, Other: Grants or contracts from any entity.; Lilly: Other: Grants or contracts from any entity.; Meiji Seika: Consultancy, Honoraria; Chugai Pharmaceutical: Honoraria; Janssen: Honoraria; AstraZeneca: Honoraria; Novartis: Honoraria; Sawai: Honoraria; Amgen: Honoraria; Asteras: Honoraria; Kissei: Honoraria; Takeda: Honoraria; Pfizer: Other: Grants or contracts from any entity.; Sanofi: Honoraria.

*signifies non-member of ASH