3 MEITL MEITL MEITL. [DOI] /j.issn Cancer Hospital, Chongqing , China

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1 Med J Chin PLA, Vol. 43, No. 1, January 1, T 3 [ ] T (MEITL) 3 MEITL T CD3 CD8 CD56 T (TIA-1) CD4 CD5 Ki-67 50%~70% 1 CHOP GDP 2 2 CHOP 1 3 MEITL MEITL [ ] T [ ] R733.4 [ ] A [ ] (2018) [DOI] /j.issn Monomorphic epitheliotropic intestinal T-cell lymphoma: A clinic-pathologic study of 3 cases and literature review WANG Li 1, HUANG De-hong 1, XIAO Chun-yan 1, LI Yu 2, ZHANG Wen-jun 1, GUO Bing-lin 1, GONG Yi 1, YANG Tao 1, LI Qi-ying 1, NAN Yin-yu 1, ZHANG Jun 3, XIANG Ying 1* 1 Department of Hematology and Oncology, Chongqing University Cancer Hospital & Chongqing Cancer Institute & Chongqing Cancer Hospital, Chongqing , 2 Department of Pathology, Chongqing Medical University, Chongqing , 3 Department of Gastrointestinal Surgery, First Affiliated Hospital of Chongqing Medical University, Chongqing , * Corresponding author: @qq.com This work was supported by the Cultivation Project of Advanced Medical Reserve Personnels of Chongqing (2017HBRC016) [Abstract] Objective To summarize the clinicopathological features of monomorphic epitheliotropic intestinal T cell lymphoma (MEITL), and provide the experiences and lessons for diagnosis and treatment. Methods The clinical manifestations, histopathological and immunohistochemical characteristics, diagnosis, treatment and prognosis of 3 cases of MEITL were analyzed retrospectively. Meanwhile, the other cases published in the literatures were reviewed and summarized. Results Two cases of middle aged and aged men and one young woman were reported in present paper. These lesions of all the 3 patients were at the small intestine, of which the female patients had skin and mammary gland involvement. The clinical manifestations were abdominal distention, mulligrubs and intestinal perforation, and the main pathological feature was the diffuse infiltration of the homogeneous tumor cells in the whole layer intestinal wall. Immunohistochemical examination showed that tumor cells expressed all T cell markers CD3, CD8, CD56, T cell cytoplasmic antigen (TIA-1), CD4 and CD5, and Ki-67 proliferation index was 50% 70%. As to the treatment, case 1 received CHOP and GDP regimens successively, and died 2 years after final diagnosis. Case 2 received CHOP regimen for 1 course of chemotherapy and died of giving up treatment. Case 3 gave up treatment, voluntary discharged and then lost. It is clear according to the previous literatures and our experiences that the MEITL possesses high invasiveness and low incidence, [ ] (2017HBRC016) [ ] [ ] ( ) ( ) ( ) [ ] @qq.com

2 and makes the diagnosis difficult; the patient's response to the current treatment is poor. Conclusions MEITL is prone to be in the small intestine. The diagnosis of MEITL should be based on clinical manifestations, pathological features and immunohistochemical detection results. MEITL is rare, the course of disease progresses rapidly, and the patients have the tendency to have intestinal perforation and poor prognosis. Early diagnosis and positive chemotherapy may lead to a better prognosis. [Key words] monomorphicepitheliatropic intestinal t-cell lymphoma; diagnosis; therapy T (enteropathy-associated T cell lymphoma EATL) T (NHL) 1% [1] EATL ( ) 10%~20% 2016 WHO EATL T (monomorphic epitheliotropic intestinal T-cell lymphoma MEITL) EATL EATL [1] MEITL 3 MEITL MEITL WHO MEITL 3 NHL 0.08% 3 4% HE En vision CD3 CD5 CD4 CD8 CD20 CD56 TIA1 CD30 Ki-67 Ki DNA-RNA EB RNA(Epsein-Barr virus encoded RNA EBER) 1.2 PubMed enteropathy associated T-cell lymphoma Monomorphic epitheliotropic intestinal T-cell lymphoma T T MEITL (CT) CHOP ( ) 1 3cm CHOP 2 GDP ( + + ) 7 6 (PR) DICE ( ) d /L X CT CHOP 1 2d CT CT ( 1A) 2

3 Med J Chin PLA, Vol. 43, No. 1, January 1, ( 1B) EBER 3 METIL CD3( 2A) CD8( 2B) CD56( 2C) TIA-1 CD4 CD5 CD20 CD79a CD30 Ki-67 50%~70%( 2D) 3 EBER EB A B 1 MEITL (HE 100) Fig. 1 Histopathological observations of MEITL tissue (HE 100) A. Diffuse infiltration in the mucosa and submucosa by lymphoma cells which are small to medium, with light cytoplasm and round anachromasis nuclei; B. Intestinal villi atrophy, crypt hyperplasia, and intraepithelial lymphocytosis A B C D 2 MEITL (En vision 100) Fig. 2 Immunohistochemistry observations of MEITL tissue (En vision 100) Tumor cells show positive expression of CD3 (A), CD8 (B) and CD56 (C). Ki-67 stain shows a cellular proliferation index of greater than 50%(D) 3 EATL MEITL The steatorrhea was first reported to be related to intestinal tumors The tumor cells were proved to be T cells Named enteropathyassociated T-cell lymphoma (EATL) 2016 WHO [1] ( 3) WHO classification named the tumor as enteropathy-type T-cell lymphoma (ETCL) Tumor cells were found to have the characteristics of tissue cells, named malignant histiocytosis of the intestine (MHI) The tumor was found to be closely related to its surrounding small intestine villus atrophy REAL classification named the tumor as intestinal T-cell lymphoma (with or without enteropathy) Type EATL (1) Type EATL (2) EATL MEITL MEITL Fig. 3 The evolution of definitions for MEITL (1)WHO classification renamed ETCL to EATL and a variant of EATL was introduced into the classification, termed the monomorphic variant of EATL or Type ; (2)WHO classification named type EATL as monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) MEITL ( 1) [2-14] [15-18] 3.1 MEITL 50 MEITL

4 MEITL Tab. 1 Global occurrence of MEITL reported in literatures Year/Reference Published location Era of diagnosis Case source Number of cases Male/Female Median of age (year) 2008 [2] Taiwan, NA 6 4/ [3] Beijing, / [4] Hong Kong, / [5] Hong Kong, Korea, Malaysia 38 25/ [6] Singapore Singapore 42/17 60 Korea, Malaysia, Australia (1 White, gender (57 Asians, 3 Whites) unknown) [7] Japan NA Japan 26 20/ [8] Singapore Singapore Malaysia, Korea, Thailand 46 (14 cases were reported in 2013) 2016 [9] Korea Korea 5 3/ [10] Japan NA Japan 4 2/ [11] Switzerland NA Switzerland, France, Germany 29 10/ [12] USA NA USA 20 18/ [13] USA NA USA, Britain Finland Canada 23 (1 Black, 9 Asians, 13 Whites) NA NA 12/ [14] Hong Kong, /4 56 NA. Data not available [14] 2 MEITL [15-18] [13] 5 20% 1 ( 2 ) ( ) [10] CD8 CD56 (MATK) T (TCR) 8q24(c-myc) MEITL T TCR TCR TCR TCR MEITL STAT5B [12] MATK MEITL EATL NK/T T [19] EATL MEITL 3.3 MEITL [3-4] MEITL T (CD3 CD56) MEITL 3 MEITL [9] MEITL [20] (PET-CT)

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