Diagnosis and Outcome of Extranodal Primary Intestinal Lymphoma in Inflammatory Bowel Disease: An ECCO CONFER Case Series

Abstract

Background There is a small but measurable increased risk of lymphoma in inflammatory bowel disease (IBD), with a suggestion that primary intestinal lymphoma in IBD is associated with inflamed tissue and immunosuppressant use, mainly thiopurines. Methods This multicentre case series was supported by the European Crohn’s and Colitis Organisation (ECCO) and performed as part of the Collaborative Network of Exceptionally Rare case reports (CONFER) project. Clinical data were recorded in a standardized case report form. Results Fifteen patients with intestinal lymphoma from 8 centres were included [12 males, 11 patients with Crohn’s disease, mean age 47.8 (±16.4 SD, range 26-76) years at lymphoma diagnosis]. Lymphoma type was diffuse large B-cell lymphoma (DLBCL) in 8, Hodgkin’s disease in 2, MALT lymphoma in 3, and single cases of immunoblastic lymphoma and indolent T cell lymphoma. Lymphoma was located within the IBD affected area in 10 patients. At lymphoma diagnosis, 9 patients had a history of azathioprine or anti-TNF use. Lymphoma was diagnosed at a mean time of 10.4 (±7.07, 1-24) years after IBD diagnosis in 11 patients, prior to IBD in 2 and concurrently in 2. Sustained remission over a median follow-up time of 6.5 (1.5-20) years was achieved in 10 patients after treatment; 5 of them had started biologic therapy (including anti-TNFs, vedolizumab and ustekinumab) for active CD subsequent to their PIL treatment. Conclusion In this small case series, two thirds of patients developed lymphoma in the IBD-affected area, and almost two thirds had a history of thiopurine or anti-TNF use. Biologics were restarted without recurrence of lymphoma in half of the remitters.

Small bowel lymphoma: clinical update and challenges for the gastroenterologist

“…A retrospective multicentre case series by ECCO looked at 15 patients with PIL in IBD. It showed a strong male predilection for PIL in patients with IBD and diffuse large B-cell lymphoma as the most common histological type [36 ▪▪ ]. It also showed that in 60% of the cases (9/15), immunosuppression was being used and intestinal inflammation was associated with PIL in two-third of the patients [36 ▪▪ ].…”

“…It showed a strong male predilection for PIL in patients with IBD and diffuse large B-cell lymphoma as the most common histological type [36 ▪▪ ]. It also showed that in 60% of the cases (9/15), immunosuppression was being used and intestinal inflammation was associated with PIL in two-third of the patients [36 ▪▪ ]. The incidence rate (per 100 000 patient-years) of PIL in the general population in 0.5 vs. 12.1 in patients with IBD.…”

Extraintestinal Cancers in Inflammatory Bowel Disease: A Literature Review

“…Primary Intestinal Lymphoma (PIL) is a rare form of NHL that primarily affects the gastrointestinal tract, specifically the small intestine [81]. While PIL can occur in individuals without any underlying conditions, there is a notable association between PIL and IBD, particularly CD [81][82][83][84]. Four case series described this association [81][82][83][84].…”

“…While PIL can occur in individuals without any underlying conditions, there is a notable association between PIL and IBD, particularly CD [81][82][83][84]. Four case series described this association [81][82][83][84]. Phillips et al reported on fifteen IBD patients with PIL treated in eight different centers [81].…”

Editorial: Pathology in the small bowel – challenges, trials and tribulations

“…The important association of lymphoma and inflammatory bowel disease is a notable concern. There is a male predilection, in 2/3 of cases the patients were on immunosuppression and the lymphoma was also associated with areas of active disease [3]. Capsule endoscopy, deviceassisted enteroscopy and imaging are complementary and have been helpful towards reaching the diagnosis of this rare entity.…”