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.
Humans ; Lymphoma, Follicular/drug therapy* ; Duodenal Neoplasms/pathology* ; Prognosis ; Tumor Microenvironment

Follicular lymphoma is an indolent malignant tumor originating from lymph nodes and lymphoid tissues, which may affect the patients' quality of survival due to the recurrence and progression. In recent years, with the deepening understand of the molecular biology and signaling pathways, many new targeted drugs for follicular lymphoma have been discovered, such as monoclonal antibodies, checkpoint inhibitors, epigenetic regulation related targeted therapies and signaling pathway inhibitors. In this review, the new progress of immunotherapy for follicular lymphoma is summarized briefly.
Antineoplastic Agents/pharmacology* ; Epigenesis, Genetic ; Humans ; Immunologic Factors/therapeutic use* ; Immunotherapy ; Lymphoma, Follicular/drug therapy*

OBJECTIVE: To investigate the efficacy and safety of bendamustine combined with gemcitabine, vinorelbine，glucocorticoids (BeGEV)±X regimen in treatment of patients with relapsed/refractory non-Hodgkin lymphoma. METHODS: A total of 18 relapsed/ refractory non-Hodgkin lymphoma patients at the age of 18 years or older hospitalized in the First People's Hospital of Changzhou from March 2020 to March 2021 were selected. They received two or more cycles of BeGEV±X regimen. X could be anti-CD20 monoclonal antibody, PD-1-blocking antibodies, lenalidomide, BTK inhibitor, Bcl-2 inhibitor and so on according to patients' disease feature. The clinical efficacy and adverse effects were observed. RESULTS: In total, 18 patients completed two or more cycles of BeGEV±X regimen, including 14 with diffuse large B-cell lymphoma, one with low-grade follicular lymphoma, one with follicular lymphoma grade 3b, one with angioimmunoblastic T-cell lymphoma and one with peripheral T-cell lymphoma, not otherwise specified. 11 patients were male. The median age of the patients was 64 years old. 17 patients had modified Ann Arbor stage Ⅲ/Ⅳ disease. 13 patients had high- intermediate risk or high risk IPI score, while 15 patients had high-intermediate high risk or high risk NCCN-IPI score. 14 cases had extranodal sites of disease. And 6 cases had bulky disease. 12 patients experienced refractory disease, while 8 patients had received 3 line or more prior treatment. After two or three cycles of chemotherapy, the complete response rate was 6/18, the partial response rate was 3/18, and the objective response rate was 9/18. From the beginning of salvage chemotherapy to the end of follow-up, the median progression-free survival time was 130 days, and the median overall survival was 152 days. The most common grade 3 to 4 adverse events were hematologic toxicities, infection and febrile neutropenia. CONCLUSION BeGEV±X is an effective salvage regimen in treatment of patients with relapsed/refractory non-Hodgkin lymphoma, while adverse events such as hematologic toxicities and infection should be closely monitored.
Humans ; Male ; Middle Aged ; Adolescent ; Female ; Lymphoma, Follicular/drug therapy*

OBJECTIVE: To evaluate the safety and efficacy of C-CAR011 in the treatment of relapsed or refractory B-cell non-Hodgkin lymphoma (R/R B-NHL) patients. METHODS: B-NHL patients treated with C-CAR011 infusion following lympho-depletion were enrolled. All the patients were followed up for 1 year after C-CAR011 treatment(5.0×10 RESULTS: The ratio of the male and female of 6 patients was 1∶1, and the patients were treated with C-CAR011 at a dose of 5.0×10 CONCLUSION C-CAR011 is a safe treatment option for R/R B-NHL; some patients could achieve long-term sustained responses after C-CAR011 infusion（ClinicalTiral.gov number, NCT03483688）.
Antigens, CD19/therapeutic use* ; Antineoplastic Combined Chemotherapy Protocols ; B-Lymphocytes ; Female ; Humans ; Lymphoma, Follicular ; Lymphoma, Large B-Cell, Diffuse ; Male ; Middle Aged ; Neoplasm Recurrence, Local/drug therapy* ; Treatment Outcome

PURPOSE: ¹⁸F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) is the standard imaging modality for response evaluation in FDG-avid lymphoma, but the prognostic value is not established in follicular lymphoma (FL). This study investigated the prognostic value of Deauville 5-point scale (D5PS) from paired interim PET/CT (PET(Interim)) and end-of-induction therapy PET/CT (PET(EOI)) in patients with FL.METHODS: FL staging and response assessment PET/CT images from 2013 to 2015 were retrospectively reviewed. PET(Interim) was performed 3 or 4 cycles after chemotherapy and PET(EOI) after 6 or 8 cycles. D5PS scores of 1, 2, and 3 were considered as negative (−), and scores 4 and 5 were considered as positive (+). Statistical analysis was done using Cox regression analysis, Kaplan-Meier survival analysis, and the log-rank test.RESULTS: Thirty-three patients with set of baseline, interim, and end-of-induction therapy PET/CTstudies were included. Ten patients (30.3%) had progression. The median progression-free survival (PFS) was 38.8 months (range 3.5–72.7 months). On PET(Interim), 23 patients were negative and 10 were positive. On PET(EOI) scans, 29 patients were negative, and 4 were positive. On multivariate analysis, PET(EOI)(−) was associated with longer PFS. PET(Interim)(+) and PET(EOI)(+) patients had a significantly shorter PFS than PET(Interim)(−) patients (39.9 months, 95%confidence interval [CI] 23.0–56.9, versus 55.5months, 95%CI 49.7–61.2, p=0.005) and PET(EOI)(−) patients (14.2 months, 95% CI 8.5–19.8, versus 60.5 months, 95% CI 52.1–69.0, p<0.001).CONCLUSION: For patients with FL, PET(Interim) and PET(EOI) response is predictive of PFS, and PET(EOI)(+) is an independent prognostic factor for progression of FL.
Disease-Free Survival ; Drug Therapy ; Electrons ; Fluorodeoxyglucose F18 ; Humans ; Kaplan-Meier Estimate ; Lymphoma ; Lymphoma, Follicular ; Multivariate Analysis ; Positron-Emission Tomography ; Positron-Emission Tomography and Computed Tomography ; Retrospective Studies

Objective: To explore the clinical characteristics of follicular lymphoma (FL) in the era of rituximab combined with chemotherapy and the prognostic significance of the follicular lymphoma international prognostic index (FLIPI), follicular lymphoma international prognostic index 2 (FLIPI2), international prognostic index (IPI), revised international prognostic index (R-IPI), National Comprehensive Cancer Network international prognostic index (NCCN-IPI) among Chinese patients. Methods: 229 FL patients who were treated initially with rituximab combined with CHOP-like (cyclophosphamide, doxorubicin, vincristine and prednisone) chemotherapy from November 2008 to April 2018 were analyzed retrospectively and all were scored by the above clinical index. Univariate and multivariate survival analysis were performed on 201 patients who completed the treatment and were followed regularly. Results: In the univariate survival analysis, age>60 years, hemoglobin<120 g/L, elevated serumβ(2)- macroglobulin, involvement of bone marrow and elevated CRP were the risk prognostic factors for overall survival (OS) and progression free survival (PFS). Moreover, the analysis of the OS and PFS between rituximab (R) maintenance (RM) group and non-maintenance (non-RM) group showed that the OS and PFS of RM group were better than those of non-RM. In the multivariate analysis of OS, hemoglobin<120 g/L, involvement of bone marrow, elevated CRP and non-RM were independent prognostic factors. In the multivariate analysis of PFS, hemoglobin<120 g/L, CRP and non-RM were independent prognostic factors. When FLIPI2 was included in the multivariate analysis, CRP and FLIPI2 were independent prognostic factors in both OS and PFS, and non-RM was independent prognostic factors in PFS. Conclusion: FLIPI2 is the better risk stratification in FL patients in the era of rituximab.
Antineoplastic Combined Chemotherapy Protocols ; Cyclophosphamide ; Disease-Free Survival ; Doxorubicin ; Humans ; Lymphoma, Follicular/drug therapy* ; Prednisone ; Prognosis ; Retrospective Studies ; Rituximab/therapeutic use* ; Vincristine

Background: Prospective real-life data on the safety and effectiveness of rituximab in Chinese patients with diffuse large B-cell lymphoma (DLBCL) or follicular lymphoma (FL) are limited. This real-world study aimed to evaluate long-term safety and effectiveness outcomes of rituximab plus chemotherapy (R-chemo) as first-line treatment in Chinese patients with DLBCL or FL. Hepatitis B virus (HBV) reactivation management was also investigated. Methods: A prospective, multicenter, single-arm, noninterventional study of previously untreated CD20-positive DLBCL or FL patients receiving first-line R-chemo treatment at 24 centers in China was conducted between January 17, 2011 and October 31, 2016. Enrolled patients underwent safety and effectiveness assessments after the last rituximab dose and were followed up for 3 years. Effectiveness endpoints included progression-free survival (PFS) and overall survival (OS). Safety endpoints were adverse events (AEs), serious AEs, drug-related AEs, and AEs of special interest. We also reported data on the incidence of HBV reactivation. Results: In total, 283 previously untreated CD20-positive DLBCL and 31 FL patients from 24 centers were enrolled. Three-year PFS was 59% (95% confidence interval [CI]: 50-67%) for DLBCL patients and 46% (95% CI: 20-69%) for FL patients. For DLBCL patients, multivariate analyses showed that PFS was not associated with international prognostic index, tumor maximum diameter, HBV infection status, or number of rituximab treatment cycles, and OS was only associated with age >60 years (P < 0.05). R-chemo was well tolerated. The incidence of HBV reactivation in hepatitis B surface antigen (HBsAg)-positive and HBsAg-negative/hepatitis B core antibody-positive patients was 13% (3/24) and 4% (3/69), respectively. Conclusions: R-chemo is effective and safe in real-world clinical practice as first-line treatment for DLBCL and FL in China, and that HBV reactivation during R-chemo is manageable with preventive measures and treatment. Trial Registration ClinicalTrials.gov, NCT01340443; https://clinicaltrials.gov/ct2/show/NCT01340443.
Aged ; Aged, 80 and over ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; China ; Cyclophosphamide ; administration & dosage ; Doxorubicin ; administration & dosage ; Female ; Follow-Up Studies ; Humans ; Lymphoma, Follicular ; drug therapy ; Lymphoma, Large B-Cell, Diffuse ; drug therapy ; Male ; Middle Aged ; Prospective Studies ; Rituximab ; therapeutic use ; Vincristine ; administration & dosage

Diagnosing tumor-induced osteomalacia is often challenging because conventional imaging modalities may fail to locate the responsible tumor. This report describes the ability of ⁶⁸Ga-DOTATOC PET/CT to successfully distinguish between the responsible phosphaturic mesenchymal tumor and concurrent lymphoma lesions. A 52-year-old man with bone pain for several years was diagnosed with a vitamin D-resistant hypophosphatemic osteomalacia. Whole body ¹⁸F-FDG PET/CT revealed multiple enlarged hypermetabolic lymph nodes in his bilateral cervical, axillary, mediastinal, abdominal, pelvic, and inguinal regions. Core needle biopsy of the right cervical lymph node confirmed the diagnosis of follicular lymphoma. However, lymphoma was not considered the cause of osteomalacia. ⁶⁸Ga-DOTATOC PET/CT before chemotherapy showed a small nodule with intensely increased uptake in the right inguinal region, which was distinguished from the other enlarged lymph nodes. The nodule was surgically removed and histopathologically consistent with phosphaturic mesenchymal tumor. After surgery, the patient's serum phosphorus and alkaline phosphatase levels normalized without nutritional supplement.
Alkaline Phosphatase ; Biopsy, Large-Core Needle ; Diagnosis ; Drug Therapy ; Humans ; Hypophosphatemia ; Lymph Nodes ; Lymphoma ; Lymphoma, Follicular ; Middle Aged ; Osteomalacia ; Phosphorus ; Positron-Emission Tomography and Computed Tomography ; Vitamins

BACKGROUND/AIMS: The Follicular Lymphoma International Prognostic Index (FLIPI) and FLIPI2 are well-known prognostic models for patients with follicular lymphoma (FL). However, their prognostic relevance has not been examined before in Korean patients with FL. METHODS: We reviewed clinical and laboratory information from our database of patients between 1995 and 2012. In total, 125 patients were stratified in three categories according to FLIPI or FLIPI2 scores: low-, intermediate-, and high-risk groups. We compared FLIPI and FLIPI2 in terms of progression-free survival (PFS) and overall survival (OS). RESULTS: Among the 125 patients, the prognostic value of FLIPI and FLIPI2 was evaluated in 73 patients who fulfilled the criteria of both prognostic models. Risk stratification by FLIPI and FLIPI2 showed significant differences in unfavorable parameters among each risk group, particularly between low- and intermediate-risk groups. The high-risk group b was significantly associated with poor PFS on both FLIPI and FLIPI2 (p < 0.05). However, the OS was significantly different only in the risk groups determined by FLIPI2 (p = 0.042). In a subgroup analysis of patients who received rituximab-containing chemotherapy, the risk stratification of both prognostic models showed a significant impact on PFS, especially in the low-risk group. CONCLUSIONS: FLIPI and FLIPI2 are appropriate prognostic models in Korean FL patients, especially for discriminating low-risk patients from intermediate- and high-risk groups.
Disease-Free Survival ; Drug Therapy* ; Humans ; Lymphoma, Follicular* ; Prognosis

Epstein-Barr virus (EBV) infection has been associated with a number of lymphoid malignancies, including endemic Burkitt's lymphoma, some classical Hodgkin's lymphoma, diffuse large B-cell lymphoma, extranodal NK/T cell lymphoma, and angioimmunoblastic T-cell lymphoma. A 59-year-old woman underwent an excisional biopsy for a left axillary mass under suspicion of malignant lymphoma. A preoperative radiological study revealed multiple enlarged lymph nodes at the left axilla, mesentery, and the left external iliac chain with hypermetabolism on a positron emission tomography-computed tomography scan. She was histologically diagnosed with EBV-positive follicular lymphoma (FL, grade 3a) and received the R-CHOP (rituximab, cyclophosphamide, hydroxydaunorubicin, vincristine, prednisone) chemotherapy regimen. Herein, we report a rare case of EBV associated FL of lymph node with its review of literature.
Axilla ; Biopsy ; Burkitt Lymphoma ; Cyclophosphamide ; Drug Therapy ; Electrons ; Female ; Herpesvirus 4, Human* ; Hodgkin Disease ; Humans ; Lymph Nodes* ; Lymphoma ; Lymphoma, B-Cell ; Lymphoma, Follicular* ; Lymphoma, T-Cell ; Mesentery ; Middle Aged ; Vincristine