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Multicenter retrospective analysis of the clinicopathologic features of monomorphic epitheliotropic intestinal T-cell lymphoma

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Abstract

Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a provisional entity in the 2017 World Health Organization classifications. To further elucidate the clinicopathologic features of this new disease, we carried out a retrospective, multicenter analysis of 42 patients with MEITL. The median age of the patients was 59 years (range, 20–84 years), and 27 patients (64 %) were male. Thirty-two patients (76 %) were Ann-Arbor stages I–II and 28 (67 %) were Lugano stages I–II1&2. The most frequent site of involvement was the jejunum (N = 21). Most cases expressed CD8 (79 %) and CD56 (95 %) and did not express CD30 (5 %) or EBER (0 %). The median progression-free survival was 6.9 months (95 % CI 4.3–9.6); the median OS was 14.8 months (2.4–27.2). Thirty-two patients (76 %) underwent surgery and 37 (88 %) received chemotherapy. A complete response (CR) rate was 38 %. Sixteen patients had undergone autologous stem cell transplantation (ASCT). Relapse or progression was documented in 24 cases, most frequently in the primary site (N = 23). Four cases showed central nervous system relapse. Age over 55 years, poor performance scale, advanced Lugano stage (IIE–IV), not achieving CR, and not receiving ASCT were associated with inferior OS. While the optimal management of MEITL remains undetermined, achieving CR and consolidative ASCT seem essential. As CHOP might be insufficient for achieving CR, more efficient combinations should be investigated. Additionally, considering the frequent local failure and CNS relapse, novel therapeutic approaches are required to improve survival.

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Authors and Affiliations

  1. Division of Hematology-Oncology, Department of Medicine, Chung-Ang University, Seoul, South Korea

    Jun Ho Yi

  2. Division of Hematology and Oncology, Department of Internal Medicine, Institute of Health Science, Gyeongsang National University Hospital, Gyeongsang National University College of Medicine, Jinju, South Korea

    Gyeong-Won Lee

  3. Division of Hematology-Oncology, Department of Medicine, Dongsan Medical Center, Keimyung University School of Medicine, Daegu, South Korea

    Young Rok Do

  4. Department of Pathology, Dongsan Medical Center, Keimyung University School of Medicine, Daegu, South Korea

    Hye Ra Jung

  5. Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea

    Jung Yong Hong, Dok Hyun Yoon & Cheolwon Suh

  6. Department of Internal Medicine, Chungnam National University College of Medicine, Daejeon, South Korea

    Yoon Seok Choi

  7. Department of Internal Medicine, Inje University Ilsan Paik Hospital, Goyang, South Korea

    Seong Yoon Yi

  8. Department of Internal Medicine, Sanggye Paik Hospital, Inje University College of Medicine, Seoul, South Korea

    Byeong Seok Sohn

  9. Department of Hemato-Oncology, Hallym University Dongtan Sacred Heart Hospital, Hwaseong, South Korea

    Byung-Su Kim

  10. Department of Internal Medicine, Dong-A University College of Medicine, Busan, South Korea

    Sung Yong Oh

  11. Division of Hematology, Department of Internal Medicine, Gil Medical Center, Gachon University College of Medicine, Incheon, South Korea

    Jinny Park

  12. Department of Hematology and Oncology, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, South Korea

    Jae-Cheol Jo

  13. Department of Pathology, Korea Cancer Center Hospital, Seoul, South Korea

    Seung-Sook Lee

  14. Department of Pathology, Hanyang University Guri Hospital, Guri-si, Gyeonggi-do, South Korea

    Young-Ha Oh

  15. Division of Hematology and Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Irwon-dong, Gangnam-gu, Seoul, 06351, South Korea

    Seok Jin Kim & Won Seog Kim

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  1. Jun Ho Yi
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Yi, J.H., Lee, GW., Do, Y.R. et al. Multicenter retrospective analysis of the clinicopathologic features of monomorphic epitheliotropic intestinal T-cell lymphoma. Ann Hematol 98, 2541–2550 (2019). https://doi.org/10.1007/s00277-019-03791-y

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