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GI NK/T Cell Lymphoma

Rahul Matnani, Karthik A Ganapathi, Suzanne K Lewis, Peter H Green, Bachir Alobeid, Govind Bhagat
Primary gastrointestinal (GI) T- and NK-cell lymphomas are usually aggressive neoplasms associated with high morbidity and mortality. Over the past two decades, however, cases of primary GI lymphoproliferative disorders (LPDs) or lymphomas of T- or NK-cell derivation with indolent behavior have been reported. These LPDs are rare and they can be challenging to diagnose as they share clinical and pathological features with both, inflammatory disorders and aggressive T- and NK-cell lymphomas. Primary, indolent clonal T-cell proliferations of the GI tract, which can be CD4+, CD8+ or CD4- CD8-, have been included as a provisional entity in the newly revised World Health Organization (WHO) classification of lymphoid neoplasms and designated 'indolent T-cell LPD of the GI tract'...
June 29, 2016: Hematological Oncology
Hideki Ishibashi, Satoshi Nimura, Kenji Ishitsuka, Yasuhito Mihashi, Mikio Mizoguchi, Shotaro Nakamura, Seiichi Okamura, Seiya Momosaki, Kunihiko Aoyagi, Shotaro Sakisaka, Morishige Takeshita
We investigated the expression of the αEβ7 integrin (CD103)-intestinal homing receptor of T-intraepithelial lymphocytes (IELs) in 130 cases of adult T-cell leukemia/lymphoma (ATLL). We detected CD103 lymphoma cells in 55% (31/56) of mainly gastrointestinal (GI)-involved ATLL cases. Among them, lymphoma cells of 18 cases located in other involved organs had similar CD103 expression patterns. Histologically, we found (a) increased reactive IELs in non-neoplastic mucosal layers in 28% (5/18) of surgical and mucosal resection cases, (b) preserved epithelial glands, and (c) numerous small intraepithelial ATLL nests in involved lesions in 36 (69%) and 21 (40%), respectively, of the 52 examined cases...
April 2016: American Journal of Surgical Pathology
Wenshuang Ding, Sha Zhao, Jianchao Wang, Qunpei Yang, Hong Sun, Jiaqi Yan, Limin Gao, Wenqing Yao, Wenyan Zhang, Weiping Liu
UNLABELLED: The gastrointestinal tract (GIT) is the most common anatomic site of extranodal non-Hodgkin lymphoma (NHL) involvement. The classification criteria of lymphoma have changed in recent decades, and few large-sample studies regarding subtype analysis of lymphoma have been performed in this site. AIM: Therefore, the present study was conducted to analyze the histological subtype distribution of the GIT. METHOD: All patients in a single institution with a diagnosis of primary NHL in the GIT were enrolled between January 2007 and April 2014...
2016: Acta Haematologica
Calogero Vetro, Giacomo Bonanno, Giorgio Giulietti, Alessandra Romano, Concetta Conticello, Annalisa Chiarenza, Paolo Spina, Francesco Coppolino, Rosario Cunsolo, Francesco Di Raimondo
Gastrointestinal lymphomas represent up to 10% of gastrointestinal malignancies and about one third of non-Hodgkin lymphomas. The most prominent histologies are mucosa-associated lymphoid tissue lymphoma and diffuse large B-cell lymphoma. However, the gastrointestinal tract can be the site of rarer lymphoma subtypes as a primary or secondary localization. Due to their rarity and the multifaceted histology, an endoscopic classification has not been validated yet. This review aims to analyze the endoscopic presentation of rare gastrointestinal lymphomas from disease diagnosis to follow-up, according to the involved site and lymphoma subtype...
August 10, 2015: World Journal of Gastrointestinal Endoscopy
Bianca Tesi, Samuel C C Chiang, Dalia El-Ghoneimy, Ayad Ahmed Hussein, Cecilia Langenskiöld, Rabia Wali, Zehra Fadoo, João Pinho Silva, Ramón Lecumberri, Sule Unal, Magnus Nordenskjöld, Yenan T Bryceson, Jan-Inge Henter, Marie Meeths
BACKGROUND: Perforin, encoded by PRF1, is a pore-forming protein crucial for lymphocyte cytotoxicity. Biallelic PRF1 nonsense mutations invariably result in early-onset hemophagocytic lymphohistiocytosis (HLH), termed familial HLH type 2 (FHL2). In contrast, biallelic PRF1 missense mutations may give rise to later-onset disease and more variable manifestations. PROCEDURE: We retrospectively searched our database for patients from families with siblings carrying biallelic PRF1 missense mutations where at least one sibling did not develop HLH, and for patients with biallelic PRF1 missense mutations and an atypical presentation of disease...
December 2015: Pediatric Blood & Cancer
Ming-Ru Wu, Tong Zhang, Albert T Gacerez, Tiffany A Coupet, Leslie R DeMars, Charles L Sentman
Substantial evidence showed that T cells are the key effectors in immune-mediated tumor eradication; however, most T cells do not exhibit antitumor specificity. In this study, a bispecific T cell engager (BiTE) approach was used to direct T cells to recognize B7H6(+) tumor cells. B7H6 is a specific ligand for the NK cell-activating receptor NKp30. B7H6 is expressed on various types of primary human tumors, including leukemia, lymphoma, and gastrointestinal stromal tumors, but it is not constitutively expressed on normal tissues...
June 1, 2015: Journal of Immunology: Official Journal of the American Association of Immunologists
M-R Wu, T Zhang, L R DeMars, C L Sentman
Chimeric antigen receptor (CAR) T-cell therapies have demonstrated durable and potentially curative therapeutic efficacy against B-cell leukemia in clinical trials. A CAR strategy can target any tumor surface antigens as long as an antigen-binding receptor can be generated. New CARs that target solid tumors and have the potential to target multiple tumor types are needed. In this study, B7H6, a ligand for the NK cell activating receptor NKp30, was targeted to create a CAR that targets multiple tumor types. B7H6 is expressed on various primary human tumors, including leukemia, lymphoma and gastrointestinal stromal tumors, but it is not constitutively expressed on normal tissues...
August 2015: Gene Therapy
Hayyam Kiratli, Salih Uzun, Aysun Yeşilirmak, Arzu Sağlam Ayhan, Figen Soylemezoğlu
PURPOSE: To report a rare case of a patient with isolated primary conjunctival extranodal natural killer/T-cell lymphoma, nasal type (ENKTCL) without nasal involvement. METHODS: The clinical course, magnetic resonance imaging, positron emission tomographic and immunohistopathological features of the patient were evaluated. RESULTS: A 57-year-old man presented with rapidly progressing swelling and redness in his right eye for 2 months. A salmon-colored mass was present under all parts of the bulbar conjunctiva...
June 2015: Cornea
Tadashi Terada
Using a computer database, the author investigated the histopathology of 10 000 consecutive gastric specimens collected in the last 12 years 2002-2013 at his pathology laboratory in a relatively large hospital in Japan. Examination of histological sections was done when appropriate. The gastric specimens were made up of 8579 benign conditions and 1421 malignant lesions. The latter comprised gastric carcinoma in 1342 cases (94.4%), gastrointestinal stromal tumor (GIST) in 34 (2.4%), mucosal-associated lymphoid tissue (MALT) lymphoma in 25 (1...
February 2016: Gastroenterology Report
Periklis G Foukas, Laurence de Leval
A large variety of lymphoma types may develop as primary intestinal neoplasms in the small intestines or, less often, in the colorectum. Among these are a few entities such as enteropathy-associated T-cell lymphoma or immunoproliferative small intestinal disease that, essentially, do not arise elsewhere than in the gastrointestinal tract. In most instances the primary intestinal lymphomas belong to entities that also occur in lymph nodes or other mucosal sites, and may show some peculiar features. In the case of follicular lymphoma, important differences exist between the classical nodal cases and the intestinal cases, considered as a variant of the disease...
January 2015: Histopathology
Jie-Yang Jhuang, Sheng-Tsung Chang, Shih-Feng Weng, Shien-Tung Pan, Pei-Yi Chu, Pin-Pen Hsieh, Chih-Hsin Wei, Shih-Cheng Chou, Chiew-Loon Koo, Chih-Jung Chen, Jeng-Dong Hsu, Shih-Sung Chuang
Extranodal natural killer (NK)/T-cell lymphoma, nasal type, is a predominantly extranodal lymphoma associated with Epstein-Barr virus occurring most frequently in the upper aerodigestive tract. There are limited reports on cellular origin and prognostic factors. We retrospectively investigated 73 cases with a median age of 54 years and a male-female ratio of 2.0:1. The upper aerodigestive tract (nasal group) was the most common site of involvement (51 cases; 70%). The other organs (n = 22; extranasal group) included the skin (12 cases; 16%) and gastrointestinal tract (5; 7%)...
February 2015: Human Pathology
Jinkala Sree Rekha, Rakhee Kar, Sajini Elizabeth Jacob, Neelaiah Siddaraju, Bettadpura Shamana Suryanarayana, Molly Mary Thabah, Rakesh Naik, Nanda Kishore Maroju
Extranodal NK/Tcell lymphomas (ENKTL) are rare, aggressive lymphomas. The most common primary site of involvement is the nasal cavity, nasopharynx and paranasal sinuses. The other sites of primary involvement are skin, gastrointestinal tract and testis. Advanced disease can show lymph node, bone marrow and peripheral blood involvement. We report a case of ENKTL of the jejunum, showing peripheral pancytopenia and haemophagocytosis in the bone marrow. The intestine showed multiple intestinal perforations, with evidence of infiltration by lymphoma with coexistent strongyloides infestation...
September 2014: Indian Journal of Hematology & Blood Transfusion
Steven H Swerdlow
With the current 2008 WHO monograph on the classification of malignant lymphomas about 6 years old, work on a significant update has begun. A meeting of the Clinical Advisory Committee was already held, where the editors, selected authors and internationally prominent clinicians held wide-ranging discussions concentrating on areas where new validated data exists that would impact on the classification or on the characterization and criteria for specific entities. Although publication of the update is not expected until Fall, 2015, areas in need of updating are established...
October 2014: Pathology
Karthik A Ganapathi, Stefania Pittaluga, Oreofe O Odejide, Arnold S Freedman, Elaine S Jaffe
There are no "benign lymphomas", a fact due to the nature of lymphoid cells to circulate and home as part of their normal function. Thus, benign clonal expansions of lymphocytes are only rarely recognized when localized. Recent studies have identified a number of lymphoid proliferations that lie at the interface between benign and malignant. Some of these are clonal proliferations that carry many of the molecular hallmarks of their malignant counterparts, such as BCL2/IGH and CCND1/IGH translocations associated with the in situ forms of follicular lymphoma and mantle cell lymphoma, respectively...
September 2014: Haematologica
Steven H Swerdlow, Elaine S Jaffe, Pierre Brousset, John K C Chan, Laurence de Leval, Philippe Gaulard, Nancy Lee Harris, Stefano Pileri, Lawrence M Weiss
The cytotoxic T-cell and natural killer (NK)-cell lymphomas and related disorders are important but relatively rare lymphoid neoplasms that frequently are a challenge for practicing pathologists. This selective review, based on a meeting of the International Lymphoma Study Group, briefly reviews T-cell and NK-cell development and addresses questions related to the importance of precise cell lineage (αβ-type T cell, γδ T cell, or NK cell), the implications of Epstein-Barr virus infection, the significance of anatomic location including nodal disease, and the question of further categorization of enteropathy-associated T-cell lymphomas...
October 2014: American Journal of Surgical Pathology
Li-Li Lou, Xi-Nan Cen, Jin-Ping Ou, Yu-Jun Dong, Ze-Yin Liang, Zhi-Xiang Qiu, Wen-Sheng Wang, Wei-Lin Xu, Yuan Li, Mang-Ju Wang, Li-Hong Wang, Yue Yin, Yu-Hua Sun, Wei Liu, Qian Wang, Ying Wang, Han-Yun Ren
This study was aimed to analyze the clinical and pathological characteristics of patients with primary extranodal lymphoma (PENL). A total of 236 patients with PENL were enrolled to evaluate the clinical and pathological features. The clinical data of 236 patients with PENL confirmed by pathological and immunohistochemical methods between January 2001 and March 2012 were analyzed retrospectively. The results indicated that: (1)236 patients with PENL accounted for 40.7% of lymphoma over the same period. Median age was 55 years old (from 16 to 91 years old) ...
February 2014: Zhongguo Shi Yan Xue Ye Xue za Zhi
Huangming Hong, Chaoyong Liang, He Huang, Chengcheng Guo, Ying Tian, Tingzhi Liu, Mengping Zhang, Xueying Li, Zhao Wang, Xiaojie Fang, Jiatian Lin, Tongyu Lin
No abstract text is available yet for this article.
November 2014: Leukemia & Lymphoma
Do Hoon Kim, Danbi Lee, Jeong Won Kim, Jooryung Huh, Seong Ho Park, Hyun Kwon Ha, Cheolwon Suh, Soon Man Yoon, Kyung-Jo Kim, Kee Don Choi, Byong Duk Ye, Jeong-Sik Byeon, Ho June Song, Hwoon Yong Jung, Suk-Kyun Yang, Jin-Ho Kim, Seung-Jae Myung
BACKGROUND AND AIM: Little is known about the clinicopathological characteristics of primary gastrointestinal T-cell lymphomas (PGITL). This study evaluated the clinical and endoscopic features of the pathological subtypes of PGITL. METHODS: Forty-two lesions in 36 patients with PGITL were assessed, including 15 enteropathy-associated T-cell lymphomas (EATL), 13 peripheral T-cell lymphomas (PTCL), 10 NK/T-cell lymphomas (NK/TL), and four anaplastic large cell lymphomas (ALCL)...
May 2014: Journal of Gastroenterology and Hepatology
Seok Jin Kim, Hyun Ae Jung, Shih-Sung Chuang, Huangming Hong, Cheng-Cheng Guo, Junning Cao, Xiao-Nan Hong, Ritsuro Suzuki, Hye Jin Kang, Jong Ho Won, Wee-Joo Chng, Yok-Lam Kwong, Cheolwon Suh, Yu-Qin Song, Jun Zhu, Kevin Tay, Soon Thye Lim, Junji Suzumiya, Tong-Yu Lin, Won Seog Kim
BACKGROUND: The gastrointestinal (GI) tract is one of the most common extranasal sites in extranodal NK/T-cell lymphoma (ENKTL). However, data regarding ENKTL involving the GI tract are relatively scarce. Thus, we performed a multicenter, multinational retrospective study to analyze clinical features and treatment outcomes of ENKTL involving the GI tract. PATIENTS AND METHODS: Patients with ENKTL involving the GI tract diagnosed in twelve participating centers between 1991 and 2012 were retrospectively analyzed from five Asian countries...
2013: Journal of Hematology & Oncology
Ming Jiang, Xinchuan Chen, Zhihui Yi, Xue Zhang, Bo Zhang, Feng Luo, Yu Jiang, Liqun Zou
BACKGROUND: The gastrointestinal tract is the most common primary site of extranodal lymphomas, whereas gastrointestinal natural killer/T-cell (GINKT) lymphomas are relatively rare. To date, neither the prognostic characteristics nor the clinical features or optimal therapeutic approach for GINKT has yet been defined. PATIENTS AND METHODS: In this study, a retrospective analysis was carried out on clinical data obtained from 47 patients diagnosed with GINKT lymphoma between May 1999 and August 2011 at West China Hospital...
September 2013: Journal of Clinical Gastroenterology
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