Objective: To explore the clinical, pathological, diagnostic, treatment, and prognostic features of children with mature B-cell lymphoma (MBCL) . Methods: This retrospective study included pediatric patients with MBCL with chromosome 11 long-arm abnormalities who were diagnosed and treated at our hospital from December 2018 to February 2023. Results: Among the 11 pediatric patients with MBCL, nine were male and two were female, with a median age of 9 (2-13) years and a median disease course of 1.8 (0.5-24) months. The clinical manifestations were cervical lymph node enlargement in four patients, nasal congestion and snoring in four patients, abdominal pain in two patients, and difficulty breathing in one patient. There were seven cases of Burkitt's lymphoma, two of follicular lymphoma, and two of advanced B-cell lymphoma according to the pathological morphology examination. No patients had central nervous system or bone marrow involvement, and no extensive metastasis was observed on B-ultrasound or positron emission tomography-computed tomography (PET/CT). One patient had a huge tumor lesion. The Revised International Pediatric Non-Hodgkin Lymphoma Staging System classified four patients as stage Ⅱ, five as stage Ⅲ, and two as stage Ⅳ. 11q probe detection showed five cases of 11q gain, three of 11q loss, and three of both gain and loss. FISH showed positive MYC expression in three patients, including eight with advanced B-cell lymphoma with 11q abnormalities and three with Burkitt's lymphoma with 11q abnormalities. According to the 2019 edition of the National Health Commission's diagnostic and treatment guidelines for invasive MBCL in children, one patient was classified as Group A, two as Group B, and eight as Group C. Early evaluation of the efficacy showed complete remission. After mid-term evaluation, the intensity of chemotherapy was reduced in Group B and Group C. Among two cases of chemotherapy, the remaining nine cases had a median follow-up of 32 (6-45) months, and none had event-related survival. Conclusion: The incidence of MBCL with 11q abnormalities in children is low, clinical symptoms are mild, and progression is slow. The absence of MYC, BCL2, BCL6 rearrangements, C-MYC negative and 11q abnormalities on FISH is an important diagnostic indicator, and reducing the intensity of chemotherapy can improve prognosis.
Humans ; Female ; Male ; Child ; Adolescent ; Burkitt Lymphoma/genetics* ; Chromosomes, Human, Pair 11 ; Positron Emission Tomography Computed Tomography ; Retrospective Studies ; Lymphoma, Follicular ; Chromosome Aberrations

Objective: To summarize the pathological diagnosis, clinical features, treatment methods and outcomes of pediatric-type follicular lymphoma (PTFL). Methods: Clinical data including the pathology, clinical features, treatment methods, and follow-up results of 9 PTFL patients admitted to Henan Cancer Hospital from February 2017 to February 2023 were analyzed retrospectively. Results: The age of onset in 9 children was 6 to 18 years, all the patients were males. The clinical manifestation was local painless lymph node enlargement in the head and neck, with a stage of Ⅰ-Ⅱ. The histomorphological characteristics of PTFL were similar to those of classic follicular lymphoma (FL). The germinal center of most follicles were enlarged, the mantle zone disappeared, centroblasts were easily visible, and the histological grade were mostly grade Ⅲ, which may be accompanied by the "starry sky" phenomenon. Monoclonal peaks can be seen in B cell clonal rearrangements (BCR). Immunohistochemistry (IHC) showed CD20 positive, CD10 positive, Bcl-6 positive, Bcl-2 negative, C-myc negative, and Ki-67 was 70%-95%. Fluorescence in situ hybridization (FISH) test was negative for t (14, 18), Bcl-2 translocation, and C-myc translocation. Six cases underwent surgical resection, and 3 cases underwent surgical resection combined with chemotherapy. Up to February 2023, with a follow-up time of 45 to 72 months, all children survived without any recurrence and were in a complete remission state. Conclusions: PTFL is mainly characterized by adolescent male onset, with early clinical manifestations and pathological manifestations of high-level histological status, high proliferation index, and lack of t (14; 18)/Bcl-2 translocation and Bcl-2 expression. It is mainly treated by localized surgical excision and has a good prognosis.
Child ; Adolescent ; Humans ; Male ; Female ; Lymphoma, Follicular/pathology* ; Lymphoma, B-Cell/pathology* ; In Situ Hybridization, Fluorescence ; Retrospective Studies ; Proto-Oncogene Proteins c-bcl-2/genetics*

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*

Humans ; Lymphoma, Follicular/pathology* ; Lymphoma, T-Cell, Peripheral

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 analyze the clinical manifestations, pathological characteristics, therapeutic effect and prognosis of diffuse large B-cell lymphoma (DLBCL) transformed from follicular lymphoma (FL). METHODS: A total of 45 inpatients were eligible for criteria of histologic transformation from FL to DLBCL from January 2010 to January 2021, and their clinical characteristics, diagnosis, treatment and efficacy were collected and analyzed retrospectively. RESULTS: Among the 45 patients, transformation occurred at diagnosis in 27 cases, during " watch and wait " phase in 7 cases and after treatment of FL in 11 cases. The median age was 56 years old (ranged from 27 to 76 years), with 23 male and 22 female. The transformations were observed in 8 cases with low-grade and 37 cases with high-grade FL. Extranodal involvement was present in 26 cases, including bone marrow infiltration in 16 cases. There were 17 cases with anemia and 32 cases met the GELF criteria of high tumor burden. B symptoms were present in 12 cases. There were 38 cases with Ki-67≥50%. The germinal center B-cell-like (GCB) subtype of DLBCL was observed in 43 cases. Efficacy was evaluated in 45 cases. The OR, CR and PR rate were 80.0%, 60.0% and 20.0%, respectively. The OR,CR rate of patients received R-CHOP was higher than those of patients received other regimens (86.11% vs 55.55%, P=0.063; 66.67% vs 33.33%, P=0.126), but there was no significant statistical difference. The early transformation (at diagnosis) group showed the highest OR rate (85.18%) and CR rate (74.07%). The median follow-up time of all patients was 26 (4-120) months, and the median PFS (2-120 months) and median OS (5-120 months) had not yet reached. The 3-year PFS and OS were 55% and 70%, respectively. In univariate analysis, factors affecting PFS includ OR rate and POD24, and factors affecting OS includ IPI score, OR rate, POD24, B symptoms and anemia (P<0.05).Multivariant analysis indicated that OR rate and POD24 were the independent prognostic factors for PFS (P<0.05) and IPI score was an independent prognostic factor for OS (P<0.05). CONCLUSION The OR and CR rates are higher in early transformation group of patients with DLBCL transformed from FL. Patients with anemia, B symptoms, POD24, and high-risk score have poor prognosis. IPI score is the independent prognosis factor for OS.
Humans ; Female ; Male ; Adult ; Middle Aged ; Aged ; Lymphoma, Follicular ; Retrospective Studies ; Lymphoma, Large B-Cell, Diffuse ; Anemia

Humans ; Lymphoma, Follicular ; Lymph Nodes

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

OBJECTIVE: To investigate the clinical characteristics and prognostic factors of primary follicular lymphoma (FL) patients with grade 3 or large B cell transformation, so as to provide more reference for the subsequent clinical diagnosis and treatment. METHODS: Forty-seven primary FL patients with grade 3 or large B cell transformation from March 2010 to March 2018 were selected, the clinical characteristics and survival of patients were analyzed. Cox regression model were used to evaluate the related prognostic factors. RESULTS: The cumulative progression-free survival rate and cumulative overall survival rate of 47 patients in 3-year follow-up reached to 55.32% (26/47) and 80.85% (38/47) respectively. There were significant differences in cumulative progression-free survival rate and cumulative overall survival rate among different subgroups of IPI, FLIPI-1 and FLIPI-2 in 3-year follow-up (P<0.05). The cumulative progression-free survival rate during follow-up for 3-year in the patients with Ann Arbor staging for III-IV stage, lymph node-involved site≥5, lymph node-involved site with diameter more than 3 cm≥3 and extranodal lesions≥3 was significantly lower than other subgroups (P<0.05). The cumulative overall survival rate in 3-year follow-up of patients with LDH levels≥240 U/ml was significantly lower than patients with LDH levels < 240 U/ml (P<0.05). Univariate analysis showed that Ann Arbor stage for III-IV, lymph node-involved site number≥5, >3 cm lymph node-involved site number≥3, extranodal lesion site number≥2, IPI score=2-3, FLIPI-1 score and FLIPI-2 score≥3 were the risk factors for progression-free survival (P<0.05); LDH≥240 U/ml, IPI score=2-3 and FLIPI-2 score≥3 were risk factors for overall survival (P<0.05). Cox regression model multivariate analysis showed that IPI score=2-3 was the independent risk factor for progression-free survival and overall survival (P<0.05). FLIPI-2 score≥3 was the independent risk factor for overall survival (P<0.05). CONCLUSION Primary FL patients with grade 3 or large B cell transformation by using the existing treatment regimen might be possibly curable, and the current treatment strategies and IPI score can be used to predict the clinical prognosis of patients.
B-Lymphocytes ; Disease-Free Survival ; Humans ; Lymphoma, Follicular ; Prognosis ; Retrospective Studies ; Risk Factors ; Survival Rate