Enteropathy-associated T-cell lymphoma: clinical and histological findings from the International Peripheral T-Cell Lymphoma Project

Delabie, Jan; Holte, Harald; Vose, Julie M.; Ullrich, Fred; Jaffe, Elaine S.; Savage, Kerry J.; Connors, Joseph M.; Rimsza, Lisa M.; Harris, Nancy L.; Müller-Hermelink, Konrad; Rüdiger, Thomas; Coiffier, Bertrand; Gascoyne, Randy D.; Berger, Françoise; Tobinai, Kensei; Au, Wing Y.; Liang, Raymond; Montserrat, Emili; Hochberg, Ephraim P.; Pileri, Stefano; Fédérico, Massimo; Nathwani, Bharat N.; Armitage, Jamés O.; Weisenburger, Dennis D.

doi:10.1182/blood-2011-02-335216

Cited by 313 publications

(352 citation statements)

References 33 publications

(49 reference statements)

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“…These results are consistent with the immunophenotype of Type II EATL as previously described. [1][2][3][4][5][6] In the present study, a high proportion of cases expressed γδ-TCR (10/20, 50%), and six cases were negative for both αβ and γδ-TCR, a finding that differs from the description in the WHO classification, which states that EATL Type II cases express αβ-TCR. Tan et al 7 reported predominant expression of αβ-TCR over γδ-TCR, and Chan et al 8 reported that 78% of Type II EATL cases expressed γδ-TCR.…”

Section: Discussioncontrasting

confidence: 89%

Genomic and immunohistochemical profiles of enteropathy-associated T-cell lymphoma in Japan

et al. 2015

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Enteropathy-associated T-cell lymphoma (EATL) is a rare primary T-cell lymphoma of the digestive tract. EATL is classified as either Type I, which is frequently associated with and thought to arise from celiac disease and is primarily observed in Northern Europe, and Type II, which occurs de novo and is distributed all over the world with predominance in Asia. The pathogenesis of EATL in Asia is unknown. We aimed to clarify the histological and genomic profiles of EATL in Japan in a homogeneous series of 20 cases. The cases were characterized by immunohistochemistry, high-resolution oligonucleotide microarray, and fluorescence in situ hybridization (FISH) at five different loci: 1q21.3 (CKS1B), 6q16.3 (HACE1), 7p22.3 (MAFK), 9q33.3 (PPP6C), and 9q34.3 (ASS1, CARD9) using formalin-fixed paraffin-embedded sections. The histological appearance of EATL ranged from medium-to large-sized cells in 13 cases (65%), small-to medium-sized cells in five cases (25%), and medium-sized in two cases (10%). The immunophenotype was CD2 + (60%), CD3e + (100%), CD4 + (10%), CD7 + (95%), CD8 + (80%), CD56 + (85%), TIA-1 + (100%), Granzyme B + (25%), T-cell receptor (TCR)β + (10%), TCRγ + (35%), TCRγδ + (50%), and double negative for TCR (six cases, 30%). All cases were EBER − . The genomic profile showed recurrent copy number gains of 1q32. 3, 4p15.1, 5q34, 7q34, 8p11.23, 9q22.31, 9q33.2, 9q34.13, and 12p13.31, and losses of 7p14.1. FISH showed 15 patients (75%) with a gain of 9q34.3 with good correlation with array comparative genomic hybridization. EATL in Japan is characterized by non-monomorphic cells with a cytotoxic CD8 + CD56 + phenotype similar to EATL Type II. The genomic profile is comparable to EATL of Western countries, with more similarity to Type I (gain of 1q and 5q) rather than Type II (gain of 8q24, including MYC). The 9q34.3 gain was the most frequent change confirmed by FISH irrespective of the cell origin of αβ-T-cells and γδ-T-cells.

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Section: Discussioncontrasting

confidence: 89%

Genomic and immunohistochemical profiles of enteropathy-associated T-cell lymphoma in Japan

et al. 2015

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“…Retrospective studies in Western populations have shown that it represents 10%-16% of all primary lymphomas of the GI tract [1,2]. In another recent international study, it accounted for 5.4% of all lymphomas [3]. EATL in its classical form is predominantly found in Western countries, especially in northern Europe.…”

Section: Introductionmentioning

confidence: 99%

“…The immunophenotype is also distinct from classical EATL, being CD3 1 , CD4 -, CD8 1 , and D56 1 . It is less common in Western countries, accounting for 10%-20% of the cases in published series of EATL [1][2][3][4][5]. It is currently undefined if type II EATL is a histologic variant of classical EATL or represents a separate lymphoma entity.…”

Section: Introductionmentioning

confidence: 99%

Type II enteropathy‐associated T‐cell lymphoma: A multicenter analysis from the Asia Lymphoma Study Group

et al. 2012

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Enteropathy-associated T-cell lymphoma (EATL) is a rare primary gastrointestinal T-cell lymphoma. A multicenter study from the Asia Lymphoma Study Group identified 38 EATL patients within a 19-year period. All cases were type II EATL. Men were affected twice as common as women, at a median age of 59 (23-89) years. None had a history of celiac disease. The sites of involvement were small bowel and stomach (5%), small bowel (63%), small and large bowel (16%), and large bowel (18%). Common presenting features were bowel perforation (34%), pain (32%), and obstruction (21%). Lymphomas showed monomorphic neoplastic lymphoid infiltrates that were CD3 1 (100%), CD56 1 (91%), TIA-1 1 (96%), CD4 -CD8 1 (63%), CD4 1 CD8 1 (19%), CD4 -CD8 -(16%), and CD4 1 CD8 -(3%). Epstein Barr virus was demonstrable in three cases. Despite chemotherapy and/or surgical resection, the overall response and complete response rates were poor at 46% and 38%. The median overall survival (OS) was 7 months and progression-free-survival (PFS) 1 month. Five patients underwent hematopoietic stem cell transplantation all were alive. Age and the prognostic index for peripheral T-cell lymphoma were not prognostically significant. Good performance status was associated with better OS (P 5 0.03), and response to initial treatment led to better OS and PFS (P < 0.001). Am. J. Hematol. 87:663-668, 2012. V

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“…In some patients, EATL presents in individuals with established CD who have previously responded well to a GFD but are now symptomatic due to developing RCD and/or classic EATL [9]. Other patients present acutely with a small bowel obstruction or perforation, and this can be seen in individuals with prior undiagnosed CD or without a history of CD (EATL Type II).…”

Section: Discussionmentioning

confidence: 99%

An unexpected deterrent in diagnosing refractory celiac disease and enteropathy-associated T-cell lymphoma: a gluten-free diet

Hussain

Hussain²,

Chatterjee

et al. 2018

Journal of Community Hospital Internal Medicine Perspectives

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Enteropathy-associated T-cell lymphoma (EATL) is a rare disease found in the small bowel and is seen most commonly in patients with refractory celiac disease (RCD). We present a case of an elderly male with celiac disease (CD) diagnosed in childhood with months of abdominal pain and diarrhea despite attempting to avoid gluten in his diet. After persistent symptoms for months, the patient was admitted for an acute abdomen and was found to have small bowel perforation due to a jejunal mass that was diagnosed as an EATL. In 2–5% of adult onset CD, serious complications such as RCD or malignancy develop. The clinical course for EATL is aggressive and generally has a poor prognosis. This case highlights the importance of early clinical suspicion for a small bowel malignancy in patients with a long-standing history of CD and acute worsening of symptoms. Early workup and diagnosis is vital in improving morbidity and mortality in patients with EATL.

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Enteropathy-associated T-cell lymphoma: clinical and histological findings from the International Peripheral T-Cell Lymphoma Project

Cited by 313 publications

References 33 publications

Genomic and immunohistochemical profiles of enteropathy-associated T-cell lymphoma in Japan

Genomic and immunohistochemical profiles of enteropathy-associated T-cell lymphoma in Japan

Type II enteropathy‐associated T‐cell lymphoma: A multicenter analysis from the Asia Lymphoma Study Group

An unexpected deterrent in diagnosing refractory celiac disease and enteropathy-associated T-cell lymphoma: a gluten-free diet

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