Primary intestinal diffuse large B-cell non-Hodgkin's lymphoma: Clinical features, management, and prognosis of 66 patients

Background: In Saudi Arabia, primary gastrointestinal non-Hodgkin's lymphom a (NHL) is common. Recently we have reported one of the largest series of p rimary gastric (PG) diffuse large B-cell lymphoma (DLCL). This has prompted the analysis of another series of patients with primary intestinal DLCL to depict the clinical features and the outcome of that disease and to compar e those with that for PG involvement. Patients and methods: The data of 66 adult patients with primary intestinal NHL having DLCL histology were retrospectively reviewed. Results: Patients had a median age of 45 years. Of 64 treated patients, 16% and 84% received single and multiple modality treatment, respectively. Sev enty-six percent, ten percent, and fourteen percent attained complete remis sion (CR), partial remission (PR), and no response/progressive disease, res pectively. Multivariate analysis failed to identify any variable that predi ct the likelihood of attaining CR. Over a median follow-up of 81 months for all 66 patients, 32 (48%) were alive and disease-free, 5 (8%) were alive w ith evidence of disease, and the remaining 29 (44%) were dead. The median o verall survival (OS) was 101 months and it was 58% (+/- 6%) and 48% (+/- 7% ) at 5- and 10-year, respectively. Of the 54 patients who achieved CR or PR , the median event-free survival (EFS) was not reached, but the predicted 5 - and 10-year EFS was 61% (+/- 7%) and 52% (+/- 7%), respectively. Only low serum albumin (< 30 g/l) was associated with adverse OS and EFS in a univa riate analysis, however, multivariate analysis was not possible. Our analys is showed that compared with single-modality management, multi-modality str ategy attained significantly higher CR, and advantageous EFS, but without a significant superior effect on OS. In comparison with patients with PG DLC L, those with primary intestinal disease demonstrated more adverse prognost ic features, but had an equivalent survival. Conclusions: This series characterized the clinico-pathologic features and outcome of patients with primary intestinal DLCL. While surgical resection in primary intestinal NHL seems beneficial, only prospective randomized stu dies can ascertain its precise role. Compared with patients with PG NHL, pa tients with primary intestinal disease had more prevalence of adverse progn ostic features.