Clinical Research Advances of Duodenal-Type Follicular Lymphoma--Review / 中国实验血液学杂志

Duodenal-type follicular lymphoma (DFL) is a unique subtype of follicular lymphoma (FL), which often involves the second portion of duodenum (descending part of duodenum). Due to its specific pathological features, such as lack of follicular dendritic cells meshwork and disappearance of activation-induced cytidine deaminase expression, DFL presents an inert clinical course and is often confined to the intestinal tract. Inflammation-related biomarkers suggest that the microenvironment may play a likely role in the pathogenesis and favorable prognosis of DFL. Since patients generally have no obvious clinical symptoms and low progression rate, the treatment regimen for DFL is mainly observation and waiting (W&W) strategy. This study will review the latest research progress of epidemiology, diagnosis, treatment and prognosis of DFL in recent years.

Linfoma folicular duodenal primario: reporte de caso y revisión de la literatura / Primary duodenal follicular lymphoma: Case report and literature review

Resumen Los linfomas primarios del tracto gastrointestinal son infrecuentes; sin embargo, son la presentación extranodal más común de los linfomas no Hodgkin. El 30 % de los linfomas no Hodgkin corresponde a linfomas foliculares y, a su vez, cerca del 10 % de los linfomas foliculares se origina en el tracto gastrointestinal. Se han descrito factores de riesgo para el desarrollo de linfomas gastrointestinales como infección por Helicobacter pylori, inmunosupresión posterior a trasplante de órganos sólidos, enfermedad inflamatoria intestinal e infección por virus de la inmunodeficiencia humana (VIH). El linfoma duodenal folicular se reconoció como una variante del linfoma folicular en 2016 según la clasificación de la Organización Mundial de la Salud (OMS), al considerar que se trata de una condición con características biológicas y clínicas particulares. Su diagnóstico suele ser incidental o se pueden presentar síntomas leves e inespecíficos. El grado histológico suele ser bajo y el curso clínico, benigno; por lo que en gran parte de los casos se ha adoptado el manejo expectante como una opción. Otras terapias con similar efectividad son la radioterapia, el uso de rituximab y la inmunoquimioterapia. No existe a la fecha suficiente evidencia para generar un protocolo único de manejo para esta patología.

Abstract Primary gastric lymphomas are rare diseases; however, they are the most common extranodal presentation of non-Hodgkin lymphomas. 30% of non-Hodgkin lymphomas correspond to follicular lymphomas and at the same time, nearly 10% of follicular lymphomas are produced in the gastrointestinal tract. Risk factors for gastric lymphomas such as Helicobacter pylori infection, immunosuppression after solid organ transplantation, inflammatory bowel disease, and human immunodeficiency virus (HIV) infection were described. Follicular duodenal lymphoma was recognized as a variant of follicular lymphoma in 2016 according to the World Health Organization (WHO) classification, considering that it is a condition with special biological and clinical characteristics. Its diagnosis is usually incidental or mild and nonspecific symptoms may occur. The histological grade is usually low, and the clinical course is benign; Therefore, in most cases, expectant treatment has been adopted as an option. Other therapies with similar effectiveness are radiotherapy, the use of rituximab, and immunochemotherapy. There is not enough evidence to date to generate a single management protocol for this pathology.

Clinical Characteristics and Survival of Primary Duodenal Lymphoma: Analysis Based on SEER Database / 胃肠病学

Background: Primary duodenal lymphoma (PDL) is a rare extranodal malignant lymphoma. Till now, there are insufficient data regarding its clinical characteristics and prognosis. Aims: To analyze the clinical characteristics and prognostic factors of PDL based on SEER database. Methods: Patients diagnosed as PDL between 1975 and 2015 were evaluated via data collected from the SEER database. The demographic, clinical, pathological features and survival time were reviewed. Survival analysis was conducted by using Kaplan-Meier method. Univariate and multivariate Cox regression analyses were applied to identify the survival predictors. Results: A total of 1 659 patients with PDL were enrolled in the study. The median age was 64 years, and the median survival time was 40 months. The overwhelming majority of PDLs were B-cell non-Hodgkin lymphomas (B-NHL), of which, follicular lymphoma (36.0%) and diffuse large B-cell lymphoma (DLBCL; 30.5%) accounting for approximately two thirds of the PDL patients. The 1-, 3-, 5-, and 10-year overall survival rates were 49.2%, 39.0%, 31.4%, and 13.8%, respectively. The prognosis of T-cell non-Hodgkin lymphoma (T-NHL) had a worse survival than B-NHL, and the prognosis of DLBCL was the worst in B-NHL. In multivariate Cox regression analysis, male gender, old age (>65 years), divorced/widowed or unmarried, T-NHL, and advanced clinical stage were identified as the independent risk factors for poor overall and tumor-specific survival in PDL patients, while diagnosed after 1997 (rituximab launch date) was an independent protective factor (all P<0.05). Conclusions: The prognosis of PDL patients is independently correlated with the gender, age, marital status, time of diagnosis, as well as the tumor pathological type and clinical stage. Early diagnosis of DLBCL and T-NHL may prolong the survival time. Immunotherapy such as rituximab may also improve the survival.