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

Background:In Saudi Arabia, primary gastrointestinalnon-Hodgkins lymphoma (NHL) is common. Recently we have reported oneof the largest series of primary gastric (PG) diffuse large B-cell lymphoma(DLCL). This has prompted the analysis of another series of patients withprimary intestinal DLCL to depict the clinical features and the outcome ofthat disease and to compare those with that for PG involvement.Patients and methods:The data of 66 adult patients with primaryintestinal NHL having DLCL histology were retrospectively reviewed.Results:Patients had a median age of 45 years. Of 64 treatedpatients, 16% and 84% received single and multiple modalitytreatment, respectively. Seventy-six percent, ten percent, and fourteenpercent attained complete remission (CR), partial remission (PR), and noresponse/progressive disease, respectively. Multivariate analysis failed toidentify any variable that predict the likelihood of attaining CR. Over amedian follow-up of 81 months for all 66 patients, 32 (48%) were aliveand disease-free, 5 (8%) were alive with evidence of disease, and theremaining 29 (44%) were dead. The median overall survival (OS) was 101months and it was 58% (· 6%) and 48% (·7%) at 5- and 10-year, respectively. Of the 54 patients who achievedCR or PR, the median event-free survival (EFS) was not reached, but thepredicted 5- and 10-year EFS was 61% (· 7%) and52% (· 7%), respectively. Only low serum albumin(<30 g/l) was associated with adverse OS and EFS in a univariate analysis,however, multivariate analysis was not possible. Our analysis showed thatcompared with single-modality management, multi-modality strategy attainedsignificantly higher CR, and advantageous EFS, but without a significantsuperior effect on OS. In comparison with patients with PG DLCL, those withprimary intestinal disease demonstrated more adverse prognostic features, buthad an equivalent survival.Conclusions:This series characterized the clinico-pathologicfeatures and outcome of patients with primary intestinal DLCL. While surgicalresection in primary intestinal NHL seems beneficial, only prospectiverandomized studies can ascertain its precise role. Compared with patients withPG NHL, patients with primary intestinal disease had more prevalence ofadverse prognostic features.