Is ALK‐gene rearrangement overlooked in primary gastrointestinal T‐cell lymphomas? About two cases

Abstract

A 41-year-old male patient with a history of ankylosing spondylitis and Crohn disease, treated with immunomodulators and disease-modifying drugs, was diagnosed with a primary intestinal T-cell lymphoma that followed a 7.5-year-course. This transmural proliferation lacked cytological characteristics of anaplastic large cell lymphoma (ALCL), and was CD8-positive, and CD30- and anaplastic lymphoma kinase (ALK)-negative by immunohistochemistry (IHC). However, ALK-gene rearrangement (ALK-gr) was detected by fluorescence in situ hybridization (FISH) in both initial and persistent disease. The possibility of indolent T-cell lymphoproliferative disease of the gastrointestinal tract with atypical features (transmural involvement) related to ALK-gr was suggested. A previous case of aggressive 'enteropathy-associated ALCL' in the context of celiac disease was recently reported, which also lacked anaplastic morphology, and where CD30 and ALK expression was incidentally demonstrated by IHC, and ALK-gr subsequently confirmed by FISH. These two recent cases represent two distinct rare entities pertaining to the group of primary intestinal T-cell lymphomas, and they both show unexpected ALK-gr. This suggests that ALK-gr has been overlooked in the group of primary intestinal T-cell lymphomas. Performing IHC and FISH tests for ALK-gr in primary gastrointestinal T-cell lymphomas might be of importance, particularly with the advancement of targeted therapy that could impact treatment and prognosis.

CD8-positive indolent T-Cell lymphoproliferative disorder of the gastrointestinal tract: A case report and review of literature

“…ITCLD-GI could be transformed into a higher-grade lymphoma[ 18 , 19 , 22 , 26 , 33 , 34 ]. However, the vast majority of CD8+ ITLPD-GI cases show an indolent and lengthy course that lasts for many years or even decades, with a chronic, persistent recurrent or spontaneous remission pattern[ 35 ].…”

Anaplastic Large-cell Lymphoma Involving Gastrointestinal Tract: A Clinicopathologic Study of 25 Cases of a Rare Tumor at a Rare Site

“…The authors emphasized the importance of performing immunohistochemistry and test for ALK gene rearrangement in all cases of primary gastrointestinal T cell lymphomas, even those lacking an anaplastic histology in view of advances in targeted therapies which could impact treatment and prognosis. 12 Zhang et al reported a primary gastric ALK-negative EBV-negative ALCL in an 82-year-old man who presented with iron deficiency anemia but without any gastrointestinal signs or symptoms. 34 Huang and Li reported a small bowel ALCL in a young HIV patient who presented with small bowel intussusception.…”

Indolent T cell lymphoproliferative disorder of the gastrointestinal tract: an uncommon case with lymph node involvement and the classic Hodgkin’s lymphoma

“…Some patients have prolonged survival even with no drug treatment, with official recommendations calling for a careful 'watch and Case Report Indolent T cell lymphoproliferative disorder of the gastrointestinal tract: an uncommon case with lymph node involvement and the classic Hodgkin's lymphoma Jie Wu 1# , Long-Guang Li 2# , Xiang-Yan Zhang 1 , Li-Li Wang 1 , Li Zhang 1 , Yu-Jing Xiao 1 , Xiao-Ming Xing 1 , Dong-Liang Lin 1 wait' approach (5)(6)(7)(8)(9)(10)(11). However, a few reports of ITCLD-GTs showed its possibility of transformation to a highergrade lymphoma (12)(13)(14)(15). In the paper by Perry et al there were several patients who had enlarged region lymph nodes but little has been establish about whether ITCLD-GTs could infiltrate these lymph nodes because of the lack of pathological examinations (9).…”