Clinicopathologic Analysis of 6 Lymphomatoid Gastropathy Cases

Abstract

Lymphomatoid gastropathy, which was first reported in 2010, is a rare NK-cell proliferation of cyCD3, CD4, CD5, CD8, CD56 phenotypes with unknown etiology. The diagnosis is challenging, as there is histopathologic similarity to malignant lymphoma. In the 2010 report on 10 cases, all lesions were located in the stomach, and all regressed without any therapy. In the present study, we analyzed 6 cases of lymphomatoid gastropathy by investigating the clinicopathologic, immunohistochemical, and molecular findings. Endoscopic and morphologic appearances of all cases were consistent with previous reports, but 2 cases showed previously unreported unique immunophenotypes of CD4CD8. Three of 6 patients underwent lower gastrointestinal examination (1 case underwent double-balloon endoscopic examination), but no patient had lesions in the lower gastrointestinal tract. No obvious difference of histology was found between the cases of CD4-CD8-typical phenotype and ones of CD4CD8 phenotype. Both cases had similar clinical behavior as the other 4 cases, implying that the spectrum of the disease is broader than initially thought. Careful clinical and endoscopic follow-up is required for the diagnosis of lymphomatoid gastropathy, and additional case studies and molecular studies are warranted to further investigate the pathophysiology of this peculiar benign mimic of lymphoma.

T- and NK-cell lymphoproliferative disorders of the gastrointestinal tract: review and update

“…86 Since then, the clinicopathological spectrum of indolent NK-cell proliferations of the GI tract have been further characterised, and labelled as NK-cell enteropathy in North American series [87][88][89] and lymphomatoid gastropathy in Japanese series. 90,91 These designations are considered to represent different manifestations of the same disease process, though it is not clear why the distribution of lesions differs between Western and Japanese reports. As the disease involves any part of the GI tract, the term 'NK-cell enteropathy' is preferred.…”

“…The disease is rare with fewer than 45 well-documented examples in the world literature. [87][88][89][90][91][92][93][94][95][96][97][98] The median age at diagnosis is 58 years, but the age range is wide (9-76 years). 87 -98 The North American series show a female predominance (male to female ratio of 0.5:1), 87 -89 while Japanese series and case reports show an equal gender distribution.…”

“…Patients may be asymptomatic 90,91,94 or present with a variety of symptoms, including abdominal pain/discomfort, reflux, constipation, diarrhoea and rectal bleeding. 87 -89,91,95 North American series of NK-cell enteropathy describe involvement of single or multiple sites of the GI tract, including the oesophagus, 96 stomach, small bowel and colon.…”

Clinicopathological analysis of primary central nervous system NK/T cell lymphoma: rare and localized aggressive tumour among extranasal NK/T cell tumours

“…Formalin‐fixed paraffin‐embedded tissue (FFPET) sections were used. Immunohistochemical and in‐situ hybridization (ISH) studies were conducted according to previously described methods . The primary antibodies used were as follows (clone): CD3ε (LN10); CD5 (4C7); CD56 (1B6), perforin (5B10; Novocastra, Newcastle Upon Tyne, UK); CD4 (1F6); CD8 (C8/144B); granzyme B (GrB‐7; Nichirei, Tokyo, Japan) and T cell‐restricted intracellular antigen (TIA)‐1 (2G9; Beckman Coulter, Brea, CA, USA).…”

“…Immunohistochemical and in-situ hybridization (ISH) studies were conducted according to previously described methods. 16 The primary antibodies used were as follows (clone): CD3e (LN10); CD5 (4C7); CD56 (1B6), perforin (5B10; Novocastra, Newcastle Upon Tyne, UK); CD4 (1F6); CD8 (C8/ 144B); granzyme B (GrB-7; Nichirei, Tokyo, Japan) and T cell-restricted intracellular antigen (TIA)-1 (2G9; Beckman Coulter, Brea, CA, USA). Primary antibodies raised against the human T cell receptor (TCR) b chain (bF1; TCR1151, 8A3) and the TCR c chain constant region (TCR1153, c3.20; Thermo Scientific, Waltham, MA, USA) were used to detect the ab and cd TCR, respectively.…”