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

被引：64

作者：

Yi, Jun Ho ^{[1
]}

Lee, Gyeong-Won ^{[2
]}

Do, Young Rok ^{[3
]}

Jung, Hye Ra ^{[4
]}

Hong, Jung Yong ^{[5
]}

Yoon, Dok Hyun ^{[5
]}

Suh, Cheolwon ^{[5
]}

Choi, Yoon Seok ^{[6
]}

Yi, Seong Yoon ^{[7
]}

Sohn, Byeong Seok ^{[8
]}

Kim, Byung-Su ^{[9
]}

Oh, Sung Yong ^{[10
]}

Park, Jinny ^{[11
]}

Jo, Jae-Cheol ^{[12
]}

Lee, Seung-Sook ^{[13
]}

Oh, Young-Ha ^{[14
]}

Kim, Seok Jin ^{[15
]}

Kim, Won Seog ^{[15
]}

机构：

[1] Chung Ang Univ, Dept Med, Div Hematol Oncol, Seoul, South Korea

[2] Gyeongsang Natl Univ, Gyeongsang Natl Univ Hosp, Inst Hlth Sci, Coll Med,Div Hematol & Oncol,Dept Internal Med, Jinju, South Korea

[3] Keimyung Univ, Dongsan Med Ctr, Dept Med, Sch Med,Div Hematol Oncol, Daegu, South Korea

[4] Keimyung Univ, Sch Med, Dongsan Med Ctr, Dept Pathol, Daegu, South Korea

[5] Univ Ulsan, Coll Med, Asan Med Ctr, Dept Oncol, Seoul, South Korea

[6] Chungnam Natl Univ, Coll Med, Dept Internal Med, Daejeon, South Korea

[7] Inje Univ, Ilsan Paik Hosp, Dept Internal Med, Goyang, South Korea

[8] Inje Univ, Sanggye Paik Hosp, Coll Med, Dept Internal Med, Seoul, South Korea

[9] Hallym Univ, Dongtan Sacred Heart Hosp, Dept Hematooncol, Hwaseong, South Korea

[10] Dong A Univ, Coll Med, Dept Internal Med, Busan, South Korea

[11] Gachon Univ, Gil Med Ctr, Dept Internal Med, Coll Med,Div Hematol, Incheon, South Korea

[12] Univ Ulsan, Ulsan Univ Hosp, Coll Med, Dept Hematol & Oncol, Ulsan, South Korea

[13] Korea Canc Ctr Hosp, Dept Pathol, Seoul, South Korea

[14] Hanyang Univ, Guri Hosp, Dept Pathol, Guri Si, Gyeonggi Do, South Korea

[15] Sungkyunkwan Univ, Samsung Med Ctr, Dept Med, Sch Med,Div Hematol & Oncol, 50 Irwon Dong, Seoul 06351, South Korea

来源：

ANNALS OF HEMATOLOGY | 2019年 / 98卷 / 11期

关键词：

Monomorphic epitheliotropic intestinal T-cell lymphoma; Mature T-cell neoplasm; Prognosis; NERVOUS-SYSTEM INVOLVEMENT; ACUTE MYELOID-LEUKEMIA; CLINICAL-FEATURES; EXPRESSION; SURVIVAL; RELAPSE; CD56;

D O I：

10.1007/s00277-019-03791-y

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：2541 / 2550

页数：10

共 34 条

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