DUODENAL‐TYPE FOLLICULAR LYMPHOMA, A RARE ENTITY: CLINICAL ASPECTS, TREATMENT AND OUTCOMES OF BRAZILIAN PATIENTS

Abstract

Introduction: Duodenal-type follicular lymphoma (DFL) is a rare entity, presented mostly as localized disease and excellent long-term outcomes. Its pathogenesis and peculiar predilection for the duodenum is not clear. Wide use of endoscopy for several reasons seems to highlight this disease and increase its diagnosis, since many patients are asymptomatic. Environment aspects, such as diet and local infections, could play a role in its clinical presentation and evolution. Methods: We evaluated 53 patients with diagnosis of DFL based on the current WHO classification on lymphoid neoplasms. Reference Brazilian centers in hematology reported their cases to this retrospective analysis. Cases were reviewed in electronic charts after ethical approval by each center. Results: The median follow-up of the whole cohort was 2.9 years (range 0.1–11). Median age at diagnosis was 58.2 y (33–85). Using the Lugano TGI classification, 75% (n = 40) were stage I. There were 2 patients with concomitant involvement of the jejunum and 1 with colonic infiltration of follicular lymphoma. Endoscopic lesions were mainly nodules (56%) and in the second portion of the duodenum (85%). Bone marrow involvement (BMi) was present in 4 cases. Close to half of the population (51%) was asymptomatic and had the diagnosis after a screening endoscopy, while 45% had some form of mild gastrointestinal symptom that preclude the exam. Only one patient had constitutional symptoms. FLIPI 0-1 in 81% of patients, with LDH elevated in 3 patients (mean of 262.8, range 116–487). Most patients were followed with regular observation (watch and wait in 60.4%), rituximab monotherapy was administered for 15 patients (28.3%) and radiotherapy for 3 (5.7%). Only 3 patients received chemotherapy (one consolidated with radiotherapy). GI symptoms were the major cause (n = 8) for starting treatment, followed by nonspecific cause in 7 cases (many described by patient anxiety). Rituximab maintenance was performed in 9 patients, 7 treated with rituximab monotherapy and 2 with rituximab plus chemotherapy. Three patients had progression of disease (1 after watch and wait, 1 treated with rituximab monotherapy and no maintenance and 1 progressed after receiving chemotherapy for advanced disease). Within these patients, 2 had BMi. Table 1 summarize the main findings. No patient died. Within the watch and wait group, most patients didn’t perform a follow up endoscopy however three patients had a spontaneous complete remission observed by endoscopy. Conclusions: This is the first cohort of Brazilian or Latin American patients with DFL published so far. Most patients were asymptomatic or with mild gastrointestinal symptoms at diagnosis. Small multiple nodules in the second portion of the duodenum were the principal appearance. Patients tended to have low FLIPI, localized disease and good prognosis. Watch and wait strategy seems safe and spontaneous CR can occur. Keyword: indolent non-Hodgkin lymphoma No conflicts of interests pertinent to the abstract.