Hodgkin lymphoma(HL)is a heterogenous lymphoproliferative disorder of B-cell origin and represents one of the most common malignancies in children and young adults.In addition to well-known underlying factors-such as ...Hodgkin lymphoma(HL)is a heterogenous lymphoproliferative disorder of B-cell origin and represents one of the most common malignancies in children and young adults.In addition to well-known underlying factors-such as Epstein-Barr virus infection-the familial aggregation demonstrated in large population studies suggested a genetic predisposition.First-degree relatives of patients with HL have an approximately threefold increased risk of developing the disease compared to the general population.These observations have recently prompted several whole-genome studies in affected families,identifying variants possibly implicated in lymphomagenesis,including alterations in DICER1(a member of the ribonuclease III family),POT1(protection of telomeres 1),KDR(kinase insert domain receptor),KLHDC8B(kelch domain-containing protein 8B),PAX5(paired box protein 5),GATA3(GATA binding protein 3),IRF7(interferon regulatory factor 7),EEF2KMT(eukaryotic elongation factor 2 lysine methyltransferase),and POLR1E(RNA polymerase I subunit E).In this article,we review current insights into the etiopathogenesis and risks of familial HL,and present case reports involving two sisters diagnosed with HL nearly 17 years apart.Recognizing the risk for first-degree relatives may potentially increase awareness of early symptoms among family members of HL patients,leading to earlier diagnosis and better outcomes.Conversely,understanding that the hereditary risk,though higher than in the general population,remains relatively low may provide reassurance for affected families.展开更多
Objectives:B-cell lymphoma 6(BCL6)is a transcriptional repressor whose overexpression is closely linked to the progression of diffuse large B-cell lymphoma(DLBCL),making it a promising therapeutic target.This study ai...Objectives:B-cell lymphoma 6(BCL6)is a transcriptional repressor whose overexpression is closely linked to the progression of diffuse large B-cell lymphoma(DLBCL),making it a promising therapeutic target.This study aims to identify a novel small molecule,synthesized via proteolysis-targeting chimeras(PROTACs),capable of degrading BCL6,thereby inhibiting DLBCL growth and providing a foundation for future preclinical studies.Methods:The expression of BCL6 in DLBCL was analyzed using The Cancer Genome Atlas(TCGA)database and the Human Protein Atlas.Western blotting assays confirmed BCL6 expression in tumor cell lines,leading to the identification of the small molecule compound DZ-865B.To evaluate DZ-865B’s in vitro efficacy,multiple assays were performed,including protein immunoblotting,immunofluorescence,reverse transcription quantitative PCR,EDU proliferation,and soft agar cloning assays.Results:TCGA analysis revealed significant overexpression of BCL6 in DLBCL(p<0.05),corroborated by immunohistological staining and western blotting.DZ-865B induced BCL6 degradation in DLBCL cell lines(OCI-LY-1 and SU-DHL-4)in a concentration-and time-dependent manner,and induced the degradation of nuclear BCL6 through the ubiquitin-proteasome pathway.Notably,DZ-865B did not alter BCL6 mRNA levels but modulated downstream gene expression,leading to the activation of apoptosis pathway proteins and inhibition of DNA synthesis,effectively suppressing DLBCL cell growth.Conclusion:This study demonstrates that the small molecule DZ-865B targets and degrades BCL6 in DLBCL cells,promoting apoptosis and inhibiting cellular proliferation.These findings highlight DZ-865B as a potential therapeutic agent for diffuse large B-cell lymphoma.展开更多
Hepatitis C virus(HCV)and hepatitis B virus(HBV)infections are increasingly recognized as significant etiological factors in the pathogenesis of B-cell non-Hodgkin’s lymphomas(B-NHLs).Epidemiological and molecular st...Hepatitis C virus(HCV)and hepatitis B virus(HBV)infections are increasingly recognized as significant etiological factors in the pathogenesis of B-cell non-Hodgkin’s lymphomas(B-NHLs).Epidemiological and molecular studies have demonstrated a consistent association between chronic viral infection and B-NHLs.Multiple pathogenic mechanisms have been implicated in lymphomagenesis,both direct and indirect,including chronic antigenic stimulation,direct infection of B cells,and viral protein-mediated oncogenic signaling,It is likely that a combination of several pathogenic conditions is required to eventually lead to the development of lymphoma.The prevalence of B-cell lymphomas among individuals with chronic HCV or HBV infection presents a complex pathogenetic scenario,given the tumor heterogeneity and variable clinical behavior,and poses therapeutic challenges,due to the partial efficacy of current treatment options.The advent of direct-acting antivirals(DAAs)for HCV and high-genetic barrier nucleos(t)ide analogues(NAs)for HBV has improved patient outcomes.In indolent HCV-associated B-NHLs,antiviral therapy with DAAs alone often achieves sustained virologic response and may lead to lymphoma regression.Conversely,aggressive subtypes like diffuse large B-cell lymphomas require combination treatment with immunochemotherapy.In the setting of HBV-associated lymphomas,antiviral prophylaxis with potent NAs(e.g.,entecavir or tenofovir)is essential to prevent HBV reactivation during rituximab-containing chemotherapy regimen.The integration of antiviral and anticancer therapies has been shown to enhance survival outcomes while mitigating hepatic toxicity.A comprehensive understanding of the biological interplay between chronic viral infection and B-cell transformation is critical for optimizing diagnostic and therapeutic strategies.Aim of this viewpoint is to provide evidence that early viral detection and prompt management remain the most effective strategies to improve survival rates and to reduce treatment-related morbidity in these patients.展开更多
Classical Hodgkin lymphoma(cHL)is characterized by rare Hodgkin/Reed-Sternberg(HRS)tumor cells that uniformly express cluster of differentiation(CD)30 molecules and orchestrate an immunosuppressive tumor microenvironm...Classical Hodgkin lymphoma(cHL)is characterized by rare Hodgkin/Reed-Sternberg(HRS)tumor cells that uniformly express cluster of differentiation(CD)30 molecules and orchestrate an immunosuppressive tumor microenvironment,making CD30 an attractive and selective therapeutic target.We summarize the biological rationale for CD30 as a therapeutic target and the preclinical and clinical evidence across major platforms:antibody-drug conjugates(brentuximab vedotin),monoclonal antibodies(including acimtamig and its combinations with Natural Killer cells),second-and third-generation chimeric antigen receptor(CAR)-T cells,and alternative modalities.Particular attention is given to standardized response assessment(IWG,Lugano,RECIL criteria),which enables appropriate cross-trial comparisons.Taken together,the data indicate that beyond the established role of brentuximab vedotin,CD30-directed CAR-T cells and bispecific antibodies demonstrate high activity in refractory cHL,especially when used with fludarabine-containing lymphodepletion,combined with programmed cell death 1(PD-1)receptor blockade as a strategy to eradicate minimal residual disease.Key challenges include durable effector-cell persistence and optimization of sequencing and combinations;notably,loss of CD30 as an escape mechanism appears uncommon.Integrating mechanistic insights into HRS biology with clinical trial data highlights strategies to enhance the efficacy,safety,and accessibility of CD30-directed immunotherapy.This review aims to provide a concise overview of CD30-targeted approaches in cHL,emphasizing therapeutic outcomes and the evolution of CAR-T technologies.展开更多
AIM:To investigate the clinical characteristics and treatment outcomes,including visual function and overall survival(OS)of patients with ocular adnexal diffuse large B-cell lymphoma(OA-DLBCL).METHODS:This retrospecti...AIM:To investigate the clinical characteristics and treatment outcomes,including visual function and overall survival(OS)of patients with ocular adnexal diffuse large B-cell lymphoma(OA-DLBCL).METHODS:This retrospective cohort study enrolled 29 patients diagnosed with OA-DLBCL based on histopathological biopsy between 2006 and 2023.Patients were stratified into two subgroups:primary OA-DLBCL(no prior history of lymphoma)and secondary OA-DLBCL(history of DLBCL at non-ocular adnexal sites).OS was defined as the time interval from OA-DLBCL diagnosis to death from any cause.Survival analysis was performed using the Kaplan–Meier method,and prognostic factors affecting OS were identified using multivariate Cox proportional hazards regression with a stepwise selection approach.RESULTS:The cohort included 24 patients with primary OA-DLBCL(13 males,11 females;mean age:61.36±18.29y)and 5 patients with secondary OA-DLBCL(2 males,3 females;mean age:50.94±18.17y).Among the primary OA-DLBCL subgroup,12 patients(50%)presented with advanced disease(Ann Arbor stage IIIE–IV),and 16 patients(66%)were classified as T4 disease according to the tumor-node-metastasis(TNM)staging system.The mean final visual acuity was 1.72±1.10 in the primary group and 0.90±1.18 in the secondary group.The 5-year OS rate for the entire cohort was 27.7%.Multivariate analysis identified five factors significantly associated with poor survival outcomes:epiphora[adjusted hazard ratio(aHR),36.95],atherosclerotic cardiovascular disease(aHR,10.08),human immunodeficiency virus(HIV)infection(aHR,12.47),M1 stage(aHR,6.99),and secondary OA-DLBCL(aHR,6.03;all P<0.05).The median OS was 1.68y for primary OA-DLBCL and 1.12y for secondary OA-DLBCL.CONCLUSION:A substantial proportion of patients with primary OA-DLBCL present with advanced-stage disease at diagnosis.Epiphora,atherosclerotic cardiovascular disease,HIV infection,M1 stage,and secondary OA-DLBCL are independent prognostic factors for poor survival outcomes.These findings emphasize the urgent need for optimized therapeutic strategies and early screening protocols to improve the management of OA-DLBCL,particularly in developing countries.展开更多
Objective:We performed a population-based analysis focusing on primary extranodal lymphoma of either testis,kidney,bladder or prostate(PGUL).Methods:We identified all cases of localized testis,renal,bladder and prosta...Objective:We performed a population-based analysis focusing on primary extranodal lymphoma of either testis,kidney,bladder or prostate(PGUL).Methods:We identified all cases of localized testis,renal,bladder and prostate primary lymphomas(PL)versus primary testis,kidney,bladder and prostate cancers within the Surveillance,Epidemiology,and End Results database(1998e2015).Estimated annual proportion change methodology(EAPC),multivariable logistic regression models,cumulative incidence plots and multivariable competing risks regression models were used.Results:The rates of testis-PL,renal-PL,bladder-PL and prostate-PL were 3.04%,0.22%,0.18%and 0.01%,respectively.Patients with PGUL were older and more frequently Caucasian.Annual rates significantly decreased for renal-PL(EAPC:5.6%;pZ0.004)and prostate-PL(EAPC:3.6%;pZ0.03).In multivariable logistic regression models,older ager independently predicted testis-PL(odds ratio[OR]:16.4;p<0.001)and renal-PL(OR:3.5;p<0.001),while female gender independently predicted bladder-PL(OR:5.5;p<0.001).In surgically treated patients,cumulative incidence plots showed significantly higher 10-year cancer-specific mortality(CSM)rates for testis-PL,renal-PL and prostate-PL versus their primary genitourinary tumors.In multivariable competing risks regression models,only testis-PL(hazard ratio[HR]:16.7;p<0.001)and renal-PL(HR:2.52;p<0.001)independently predicted higher CSM rates.Conclusion:PGUL rates are extremely low and on the decrease in kidney and prostate but stable in testis and bladder.Relative to primary genitourinary tumors,PGUL are associated with worse CSM for testis-PL and renal-PL but not for bladder-PL and prostate-PL,even after adjustment for other-cause mortality.展开更多
BACKGROUND Mantle cell lymphoma(MCL)is an aggressive B-cell non-Hodgkin lymphoma that rarely presents with cutaneous involvement,which typically occurs in advanced disease stages.Primary cutaneous manifestations are p...BACKGROUND Mantle cell lymphoma(MCL)is an aggressive B-cell non-Hodgkin lymphoma that rarely presents with cutaneous involvement,which typically occurs in advanced disease stages.Primary cutaneous manifestations are particularly uncommon and frequently misdiagnosed.We report a diagnostically challenging case of MCL that first appeared as a nodule on the lower leg.CASE SUMMARY An elderly female had a painless red nodule on the front of her right lower leg for six months.Positron emission tomography-computed tomography showed a soft tissue mass at the same site and increased activity in the right groin lymph nodes.Skin and lymph node biopsies,along with immunostaining,confirmed MCL.The patient began combination chemotherapy,resulting in a marked improvement of skin lesions and lymphadenopathy after two weeks.CONCLUSION Cutaneous manifestations,though uncommon,may serve as the initial clinical presentation of MCL.Dermatologists and pathologists should maintain heigh-tened awareness of this diagnostic possibility when evaluating persistent,atypical cutaneous nodules,particularly in elderly patients.Histopathological evaluation with complete immunophenotypic profiling is paramount for accurate diagnosis.展开更多
BACKGROUND The concept of“duodenal-type follicular lymphoma(FL)”has already been established as a disease entity.On the other hand,because FL that develops in the colorectum is a rare disease,its clinical characteri...BACKGROUND The concept of“duodenal-type follicular lymphoma(FL)”has already been established as a disease entity.On the other hand,because FL that develops in the colorectum is a rare disease,its clinical characteristics,including its treatment and outcome,are poorly understood.AIM To clarify the characteristic clinical features of colorectal FL.METHODS We identified 5 patients with FL from December 2010 to July 2022 whose colorectal lesions were confirmed by endoscopic biopsy at our hospital and retrospectively examined their clinical features,including endoscopic findings,treatment,and long-term outcomes.RESULTS The median age of the patients was 70 years(range 62-74 years,3 males and 2 females),and 2 patients were considered to have systemic FL lesions.Endoscopic findings revealed elavated lesions in all 5 patients(polypoid 3,flat elavated 1,papular 1).Only 1 patient underwent therapeutic intervention,and 4 patients chose watchful waiting.Except for 1 patient in which the lesion spontaneously regressed,the disease recurred or progressed in 4 patients,3 of whom eventually achieved near complete remission with the administration of rituximab and bendamustine(±polatuzumab vedotin).The 5 patients in this study were still alive or exhibited long-term survival before death;the survival time ranged from 8-29 years after the onset of lymphoma.CONCLUSION In the present study,colorectal FL progressed slowly,and overall survival was good because it was sensitive to anticancer drugs even after the disease progressed without initial therapeutic intervention.展开更多
BACKGROUND Primary cardiac lymphoma(PCL)is a rare subset of cardiac tumors,often diagnosed late due to nonspecific symptoms.It predominantly affects immunocompromised individuals,primarily in the pericardium and right...BACKGROUND Primary cardiac lymphoma(PCL)is a rare subset of cardiac tumors,often diagnosed late due to nonspecific symptoms.It predominantly affects immunocompromised individuals,primarily in the pericardium and right heart.Late diagnosis mimics common cardiac ailments,leading to poor prognosis.AIM To systematically review the efficacy of rituximab in treating PCL either alone or in various chemotherapeutic regimens.Secondary objectives include evaluating morphological subtypes,assessing treatment regimens,and analyzing outcomes focusing on remission and adverse events.METHODS Following PRISMA guidelines,a comprehensive literature search was conducted across multiple databases,including PubMed,Hinari,Web of Science,and Scopus.English-language studies reporting the use of rituximab in treating PCL in humans were included.Study selection involved initial screening of titles and abstracts followed by full-text examination and data extraction.RESULTS Thirty-three case reports involving 36 patients were included in this systematic review.Diffuse large B-cell lymphoma was the predominant morphological subtype observed.The rituximab,cyclophosphamide,doxorubicin,oncovin,and prednisolone regimen emerged as the most commonly employed treatment strategy,indicating widespread acceptance and efficacy in PCL management.Combination therapies,including surgical intervention,showed promise in achieving complete remission,while some studies reported mortality despite aggressive treatment approaches.CONCLUSION Rituximab,particularly in combination with chemotherapy regimens,represents a significant advancement in PCL management,offering hope for improved patient outcomes.However,challenges such as variable treatment responses and adverse events underscore the complexity of managing PCL.Further research is warranted to refine therapeutic strategies and enhance diagnostic approaches for this rare cardiac malignancy.展开更多
BACKGROUND T/histiocyte-rich large B-cell lymphoma(T/HRBCL)is a highly aggressive subtype of diffuse large B-cell lymphoma characterized histologically by the presence of a few neoplastic large B cells amidst an abund...BACKGROUND T/histiocyte-rich large B-cell lymphoma(T/HRBCL)is a highly aggressive subtype of diffuse large B-cell lymphoma characterized histologically by the presence of a few neoplastic large B cells amidst an abundant background of reactive T lymphocytes and/or histiocytes.T/HRBCL commonly affects the lymph nodes,followed by extranodal sites,such as the spleen,liver,and bone marrow,with rare occurrences in the gastrointestinal tract.Primary gastrointestinal T/HRBCL lacks specific clinical and endoscopic manifestations,and it is difficult to differentiate from inflammatory diseases,nodular lymphocyte predominant Hodgkin lymphoma,and other diseases on a histological basis,thereby hindering early diagnosis.CASE SUMMARY A 63-year-old man was hospitalized with a one-month history of jaundice and weight loss of approximately 3 kg.Laboratory tests revealed increased hepatic parameters in a cholestatic pattern and elevated carbohydrate antigen 19-9 levels.An abdominal computed tomography scan revealed a low-density mass within the descending duodenum and dilation of the bile and pancreatic ducts.He was clinically diagnosed with a duodenal tumor.During surgery,a 7.0 cm×8.0 cm mass was identified within the descending duodenum,so pancreaticoduodenectomy and cholecystectomy were performed.Following operative biopsy,the tumor was diagnosed as primary duodenal T/HRBCL.The patient refused postoperative chemotherapy and died four months after surgery.CONCLUSION Primary duodenal T/HRBCL is an extremely rare and highly aggressive malignancy.The initial treatment strategies should be based on the original site of the tumor,the disease stage,and the patient's physical condition.Chemotherapy-based comprehensive treatment is still the main treatment method for primary gastrointestinal T/HRBCL.展开更多
Background:Primary bone marrow diffuse large B-cell lymphoma(PBM-DLBCL)represents an uncommon yet clinically aggressive hematologic malignancy.Despite its significant clinical impact,this entity lacks standardized dia...Background:Primary bone marrow diffuse large B-cell lymphoma(PBM-DLBCL)represents an uncommon yet clinically aggressive hematologic malignancy.Despite its significant clinical impact,this entity lacks standardized diagnostic criteria in current WHO classifications.Methods:We performed a retrospective analysis of 55 PBM-DLBCL cases from our institutional database and published literature(2001–2022)to characterize disease features and identify prognostic factors,with particular focus on assessing how different treatment regimens influence therapeutic efficacy and long-term outcomes.Results:The data suggested a potential link between international prognostic index(IPI)scores and poorer survival,albeit without conclusive statistical evidence(p 0.05).Treatment=response emerged as a significant prognostic factor,and patients with complete response(CR)demonstrating superior survival in Cox univariate and multivariate analysis(p 0.001).Intensive therapeutic regimens were associated with<improved clinical outcomes compared to conventional therapies.While incorporating rituximab into conventional chemotherapy regimens has demonstrated superior clinical outcomes compared to chemotherapy alone in PBM-DLBCL patients.Conclusion:Our findings highlight the aggressive nature of PBM-DLBCL and underscore the importance of early recognition,risk stratification,and optimized treatment selection for this rare disease entity.展开更多
Because human immunodeficiency virus(HIV)-associated Burkitt lymphoma(BL)has a poor prognosis new therapeutic approaches need to be developed1.Axicabtagene ciloleucel(axi-cel)is an anti-CD19 CAR-T cell commercially av...Because human immunodeficiency virus(HIV)-associated Burkitt lymphoma(BL)has a poor prognosis new therapeutic approaches need to be developed1.Axicabtagene ciloleucel(axi-cel)is an anti-CD19 CAR-T cell commercially available FDA-approved product for patients with relapsed or refractory(R/R)large B-cell lymphoma(LBCL).However,axi-cel has not been approved by the FDA for use in patients with R/R BL.展开更多
BACKGROUND Mucosa-associated lymphoid tissue(MALT)lymphoma is a subtype of extranodal marginal zone lymphoma,typically occurring in mucosal sites such as the stomach,salivary glands,and lungs.This study aims to analyz...BACKGROUND Mucosa-associated lymphoid tissue(MALT)lymphoma is a subtype of extranodal marginal zone lymphoma,typically occurring in mucosal sites such as the stomach,salivary glands,and lungs.This study aims to analyze the demographic and clinicopathologic characteristics of patients with gastric MALT lymphoma in the United States and evaluate the interaction between age and gender on survival outcomes.AIM To analyze the demographic and clinicopathologic characteristics of patients with gastric MALT lymphoma in the United States and evaluate the interaction between age and gender on survival outcomes.METHODS A retrospective cohort study was conducted using the Surveillance,Epidemiology,and End Results(SEER)database,which included 2453 patients diagnosed with MALT lymphoma from 2010 to 2021.Data were analyzed for demographic factors,tumor characteristics,treatment modalities,and survival outcomes.A Cox proportional hazards regression model was used to identify predictors of overall mortality and cancer-specific mortality.RESULTS The study predominantly included Non-Hispanic White patients(62.78%),with nearly equal gender distribution(50.31%females,49.69%males),and most diagnoses occurring in individuals aged 60-79 years.The majority of tumors were localized(80.07%).Multivariate analysis identified older age,male gender,advanced tumor stage,and socioeconomic factors—such as annual income and marital status—as independent predictors of mortality.No significant interaction between age and gender on mortality outcomes was observed.CONCLUSION Sociodemographic factors,including advanced age,male gender,annual income,and marital status,as well as advanced tumor stage,significantly impacted survival outcomes in patients with MALT lymphoma.Radiotherapy was associated with a reduction in overall mortality.Early detection is crucial for optimizing outcomes,as localized disease responds well to available treatment modalities.展开更多
BACKGROUND Primary testicular lymphoma(PTL)is a rare,aggressive malignancy,representing a small fraction of testicular tumors and non-Hodgkin lymphomas,yet it is the most common testicular malignancy in older men.Diff...BACKGROUND Primary testicular lymphoma(PTL)is a rare,aggressive malignancy,representing a small fraction of testicular tumors and non-Hodgkin lymphomas,yet it is the most common testicular malignancy in older men.Diffuse large B-cell lymphoma(DLBCL),which is typically the aggressive subtype,dominates PTL and shows diffuse B-cell infiltration.Venous tumor thrombus,uncommon in lymphomas,is uniquely reported in this case of testicular DLBCL with gonadal vein involvement.CASE SUMMARY A 62-year-old man presented with a two-month history of painless left testicular swelling and stiffness.Diagnostic imaging[ultrasonography,computed tomography(CT),and 18F-fluorodeoxyglucose positron emission tomography/CT(18FFDG-PET/CT)]revealed bilateral testicular masses and a gonadal vein tumor thrombus(SUVmax 16.5).Left orchiectomy confirmed DLBCL with CD20,Bcl-2,and MUM1 positivity(Ki-67:approximately 80%).The disease was staged as Ann Arbor stage IVA(International Prognostic Index score 3,high-intermediate risk).The patient received Rituximab,Polatuzumab Vedotin,Cyclophosphamide,Epirubicin,and Prednisolone chemotherapy,completing the first cycle with good tolerability.No adverse events were reported,and follow-up is ongoing to assess long-term outcomes.This case highlights the diagnostic utility of 18F-FDGPET/CT and the importance of multidisciplinary management in rare PTL presentations with tumor thrombus.CONCLUSION This case demonstrates the diagnostic complexities of PTL with gonadal vein tumor thrombus,underscoring the importance of considering lymphoma in elderly patients with testicular masses and venous involvement.A multi-disciplinary team including urologists,hematologists,and radiation oncologists is needed to ensure appropriate therapy.展开更多
BACKGROUND In addition to nodal lesions,over 30%of mantle cell lymphomas(MCLs)also have gastrointestinal involvement,characteristically presenting as multiple lymphomatous polyposis(MLP),which rarely involve the esoph...BACKGROUND In addition to nodal lesions,over 30%of mantle cell lymphomas(MCLs)also have gastrointestinal involvement,characteristically presenting as multiple lymphomatous polyposis(MLP),which rarely involve the esophagus.Most related papers have been case reports,and no comprehensive studies have been conducted;thus,the actual clinical situation has remained unknown for a long time.AIM To elucidate the actual clinical situation of esophageal involvement of MCL presenting with MLP,including its prognosis.METHODS From January 2001 to December 2021,among MCL patients whose gastrointestinal lesions were histopathologically confirmed by endoscopic biopsy at our center,6 patients with MLP in the esophagus were selected.We retro-spectively examined the clinical features of these patients,including their prognosis.RESULTS In all patients,multiple lesions were present in the gastrointestinal tract other than the esophagus and in the lymph nodes throughout the body,and most patients also had lesions involving the bone marrow or spleen.Most of the treatments include chemotherapy,with a 50%survival period of less than 2 years and a 5-year survival rate of approximately 30%,indicating a poor prognosis.CONCLUSION Patients with esophageal involvement of the MCL who presented with MLP had a large tumor burden and poor survival.展开更多
Fc Receptor-Like 1(FCRL1),a member of the FCRL family,contains two immunoreceptor tyrosinebased activation motifs(ITAMs)in its cytoplasmic domain and plays a critical role in B-cell biology.Its expression begins in pr...Fc Receptor-Like 1(FCRL1),a member of the FCRL family,contains two immunoreceptor tyrosinebased activation motifs(ITAMs)in its cytoplasmic domain and plays a critical role in B-cell biology.Its expression begins in pre-B-cells,dynamically shifts during B-cell development,and contributes to the regulation of human B-cell activation.Notably,FCRL1 is overexpressed in subsets of naive and memory B-cells,as well as in malignant B-cells,including those in diffuse large B-cell lymphoma(DLBCL),an aggressive and often treatment-resistant hematological malignancy.Among FCRL family members,FCRL1 stands out as a promising immunotherapeutic target due to its selective expression in malignant B-cells and its functional role in proliferation.Given the limited efficacy of current therapies for relapsed/refractory DLBCL,targeting FCRL1 could address an unmet clinical need by offering a novel,mechanism-based approach to modulate B-cell signaling and enhance anti-tumor immunity.This mini-review highlights the therapeutic potential of FCRL1-directed strategies,supporting their further exploration in preclinical models and future clinical trials for DLBCL and other B-cell malignancies.展开更多
AIM: To evaluate the endoscopic manifestations and prognoses of gastrointestinal (GI) mantle cell lymphoma (MCL). METHODS: A database search at the Department of Pathology of Okayama University Graduate School of Medi...AIM: To evaluate the endoscopic manifestations and prognoses of gastrointestinal (GI) mantle cell lymphoma (MCL). METHODS: A database search at the Department of Pathology of Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences revealed 57 MCL patients with GI involvement. Clinical records were available for 35 of the 57 patients from 21 institutions, and those 35 patients were enrolled in this study. We summarized the gross types of endoscopic features, event-free survival (EFS), and overall survival (OS) of those patients.RESULTS: Of the 35 patients, GI involvement in the esophagus, stomach, and duodenum was found in 2 (5.7%), 26 (74.3%), and 12 (34.3%) patients, respectively. Twenty-one of the 35 patients underwent colonoscopy; among them, GI involvement in the ileum, cecum, colon, and rectum was found in 10 (47.6%), 3 (14.3%), 12 (57.1%), and 10 (47.6%), respectively. Various lesions, such as superficial, protruded, fold thickening, or ulcerative, were found in the stomach, whereas multiple lymphomatous polyposis (MLP) was dominant from the duodenum to the rectum. Twelve patients were treated with a hyper-CVAD/MA regimen, and they had better OS (3-year rate, 88.3% vs 46.4%, P < 0.01) and better EFS (3-year rate, 66.7% vs 33.8%, P < 0.05) than the remaining 23 patients who were not treated with this regimen. CONCLUSION: MLP was a representative form of intestinal involvement, whereas a variety of lesions were found in the stomach. The hyper-CVAD/MA regimen may improve survival in these patients.展开更多
BACKGROUND Primary central nervous system lymphoma(PCNSL)is a non-Hodgkin lymphoma that originates in the central nervous system(CNS)and is exclusively limited to the CNS.Although most PCNSLs are diffuse large B-cell ...BACKGROUND Primary central nervous system lymphoma(PCNSL)is a non-Hodgkin lymphoma that originates in the central nervous system(CNS)and is exclusively limited to the CNS.Although most PCNSLs are diffuse large B-cell lymphomas,primary CNS T-cell lymphomas(PCNSTLs)are rare.PCNSTLs typically demonstrate some degree of enhancement on contrast-enhanced magnetic resonance imaging(MRI).To the best of our knowledge,non-enhancing PCNSTL has not been reported previously.CASE SUMMARY A 69-year-old male presented to the neurology department with complaints of mild cognitive impairment and gradual onset of left lower leg weakness over a span of two weeks.Initial MRI showed asymmetric T2-hyperintense lesions within the brain.No enhancement was observed on the contrast-enhanced T1 image.The initial diagnosis was neuro-Behçet’s disease.Despite high-dose steroid therapy,no alterations in the lesions were identified on initial MRI.The patient’s symptoms deteriorated further.An MRI performed one month after the initial scan revealed an increased lesion extent.Subsequently,brain biopsy confirmed the diagnosis of PCNSTL.The patient underwent definitive combined chemoradiotherapy.However,the patient developed bacteremia and died of septic shock approximately three months after diagnosis.CONCLUSION The absence of enhancement in the lesion did not rule out PCNSTL.A biopsy approach is advisable for pathological confirmation.展开更多
BACKGROUND Primary gastrointestinal lymphoma(PGIL)is a relatively uncommon clinical entity,exhibiting distinctive features including occult primary sites,nonspecific clinical presentations,and considerable diagnostic ...BACKGROUND Primary gastrointestinal lymphoma(PGIL)is a relatively uncommon clinical entity,exhibiting distinctive features including occult primary sites,nonspecific clinical presentations,and considerable diagnostic and therapeutic difficulties.Consequently,comprehensive clinical investigations into its clinicopathological characteristics and surgical intervention value are warranted to enhance dia-gnostic and therapeutic proficiency.AIM To investigate the clinicopathological characteristics and surgical significance of PGIL from a surgical perspective,providing a theoretical basis for optimizing diagnostic and therapeutic strategies.METHODS This study included 50 cases of PGIL treated by the General Surgery Department of the Chinese PLA Air Force Medical Center from June 2001 to March 2025.Data were extracted from the Electronic Medical Record system for retrospective analysis.A retrospective analysis was conducted on their epidemiological,clinical manifestations,imaging,pathological features,and treatment outcomes.Descriptive statistics were applied for data summarization,with continuous variables presented as frequencies and percentages.Correlations between variables were assessed using the Spearman rank correlation coefficient.RESULTS All cases had the gastrointestinal tract as the primary site.Abdominal pain was the most common initial symptom(52.0%),with 80.0%of patients experiencing pain during the course of the disease,and 38.0%experiencing hema-tochezia/melena or anemia.Computed tomography diagnosis exhibited a high overall sensitivity(94.3%);the en-doscopic detection rate was 91.5%.Diffuse large B-cell lymphoma was the most common subtype(52.0%).The im-provement rate was higher in the surgery combined with chemotherapy group than in the chemotherapy only group.The incidence of postoperative complications was 26.5%,all occurring in patients with tumors>5 cm.CONCLUSION Diffuse large B-cell lymphoma is the primary PGIL subtype.Imaging and endoscopic biopsy are diagnostic es-sentials.Surgery aids in resection,complication management,and pathologic diagnosis.Multidisciplinary,indi-vidualized strategies are recommended,necessitating further prospective molecular studies.展开更多
BACKGROUND Monomorphic epitheliotropic intestinal T-cell lymphoma(MEITL)is an uncom-mon and highly aggressive form of lymphoma that represents less than 1%of all non-Hodgkin’s lymphomas.At present,few reports have fo...BACKGROUND Monomorphic epitheliotropic intestinal T-cell lymphoma(MEITL)is an uncom-mon and highly aggressive form of lymphoma that represents less than 1%of all non-Hodgkin’s lymphomas.At present,few reports have focused on the imaging findings of MEITL,which poses significant challenges for clinical diagnosis.A 78-year-old female with recurrent vomiting and abdominal distension was admitted to our hospital.Magnetic resonance and^(18)F-fluorodeoxyglucose positron emission tomography/computed tomography(^(18)F-FDG PET/CT)examinations revealed multiple segmental malignant tumors in the small intestine with me-senteric lymph node metastasis.An endoscopic biopsy revealed MEITL.After three cycles of reduced-dose cyclophosphamide,vinorelbine,and prednisone che-motherapy,follow-up^(18)F-FDG PET/CT demonstrated a partial response to treatment.The patient was still alive after 6 months of follow-up.CONCLUSION Magnetic resonance imaging serves as a valuable tool in detecting malignant tumor lesions of MEITL,whereas^(18)F-FDG PET/CT offers additional assistance in tumor staging and assessing treatment efficacy.展开更多
文摘Hodgkin lymphoma(HL)is a heterogenous lymphoproliferative disorder of B-cell origin and represents one of the most common malignancies in children and young adults.In addition to well-known underlying factors-such as Epstein-Barr virus infection-the familial aggregation demonstrated in large population studies suggested a genetic predisposition.First-degree relatives of patients with HL have an approximately threefold increased risk of developing the disease compared to the general population.These observations have recently prompted several whole-genome studies in affected families,identifying variants possibly implicated in lymphomagenesis,including alterations in DICER1(a member of the ribonuclease III family),POT1(protection of telomeres 1),KDR(kinase insert domain receptor),KLHDC8B(kelch domain-containing protein 8B),PAX5(paired box protein 5),GATA3(GATA binding protein 3),IRF7(interferon regulatory factor 7),EEF2KMT(eukaryotic elongation factor 2 lysine methyltransferase),and POLR1E(RNA polymerase I subunit E).In this article,we review current insights into the etiopathogenesis and risks of familial HL,and present case reports involving two sisters diagnosed with HL nearly 17 years apart.Recognizing the risk for first-degree relatives may potentially increase awareness of early symptoms among family members of HL patients,leading to earlier diagnosis and better outcomes.Conversely,understanding that the hereditary risk,though higher than in the general population,remains relatively low may provide reassurance for affected families.
基金supported by the National Natural Science Foundation of China(82260716)the Key Research and Development Program of Ningxia(2023BEG02010).
文摘Objectives:B-cell lymphoma 6(BCL6)is a transcriptional repressor whose overexpression is closely linked to the progression of diffuse large B-cell lymphoma(DLBCL),making it a promising therapeutic target.This study aims to identify a novel small molecule,synthesized via proteolysis-targeting chimeras(PROTACs),capable of degrading BCL6,thereby inhibiting DLBCL growth and providing a foundation for future preclinical studies.Methods:The expression of BCL6 in DLBCL was analyzed using The Cancer Genome Atlas(TCGA)database and the Human Protein Atlas.Western blotting assays confirmed BCL6 expression in tumor cell lines,leading to the identification of the small molecule compound DZ-865B.To evaluate DZ-865B’s in vitro efficacy,multiple assays were performed,including protein immunoblotting,immunofluorescence,reverse transcription quantitative PCR,EDU proliferation,and soft agar cloning assays.Results:TCGA analysis revealed significant overexpression of BCL6 in DLBCL(p<0.05),corroborated by immunohistological staining and western blotting.DZ-865B induced BCL6 degradation in DLBCL cell lines(OCI-LY-1 and SU-DHL-4)in a concentration-and time-dependent manner,and induced the degradation of nuclear BCL6 through the ubiquitin-proteasome pathway.Notably,DZ-865B did not alter BCL6 mRNA levels but modulated downstream gene expression,leading to the activation of apoptosis pathway proteins and inhibition of DNA synthesis,effectively suppressing DLBCL cell growth.Conclusion:This study demonstrates that the small molecule DZ-865B targets and degrades BCL6 in DLBCL cells,promoting apoptosis and inhibiting cellular proliferation.These findings highlight DZ-865B as a potential therapeutic agent for diffuse large B-cell lymphoma.
基金supported by the National Italian Research Council(CNR)“Progetto DSB.AD007.305.001”to Monica Rinaldi。
文摘Hepatitis C virus(HCV)and hepatitis B virus(HBV)infections are increasingly recognized as significant etiological factors in the pathogenesis of B-cell non-Hodgkin’s lymphomas(B-NHLs).Epidemiological and molecular studies have demonstrated a consistent association between chronic viral infection and B-NHLs.Multiple pathogenic mechanisms have been implicated in lymphomagenesis,both direct and indirect,including chronic antigenic stimulation,direct infection of B cells,and viral protein-mediated oncogenic signaling,It is likely that a combination of several pathogenic conditions is required to eventually lead to the development of lymphoma.The prevalence of B-cell lymphomas among individuals with chronic HCV or HBV infection presents a complex pathogenetic scenario,given the tumor heterogeneity and variable clinical behavior,and poses therapeutic challenges,due to the partial efficacy of current treatment options.The advent of direct-acting antivirals(DAAs)for HCV and high-genetic barrier nucleos(t)ide analogues(NAs)for HBV has improved patient outcomes.In indolent HCV-associated B-NHLs,antiviral therapy with DAAs alone often achieves sustained virologic response and may lead to lymphoma regression.Conversely,aggressive subtypes like diffuse large B-cell lymphomas require combination treatment with immunochemotherapy.In the setting of HBV-associated lymphomas,antiviral prophylaxis with potent NAs(e.g.,entecavir or tenofovir)is essential to prevent HBV reactivation during rituximab-containing chemotherapy regimen.The integration of antiviral and anticancer therapies has been shown to enhance survival outcomes while mitigating hepatic toxicity.A comprehensive understanding of the biological interplay between chronic viral infection and B-cell transformation is critical for optimizing diagnostic and therapeutic strategies.Aim of this viewpoint is to provide evidence that early viral detection and prompt management remain the most effective strategies to improve survival rates and to reduce treatment-related morbidity in these patients.
基金supported by the Kazan Federal University Strategic Academic Leadership Program(PRIORITY-2030).
文摘Classical Hodgkin lymphoma(cHL)is characterized by rare Hodgkin/Reed-Sternberg(HRS)tumor cells that uniformly express cluster of differentiation(CD)30 molecules and orchestrate an immunosuppressive tumor microenvironment,making CD30 an attractive and selective therapeutic target.We summarize the biological rationale for CD30 as a therapeutic target and the preclinical and clinical evidence across major platforms:antibody-drug conjugates(brentuximab vedotin),monoclonal antibodies(including acimtamig and its combinations with Natural Killer cells),second-and third-generation chimeric antigen receptor(CAR)-T cells,and alternative modalities.Particular attention is given to standardized response assessment(IWG,Lugano,RECIL criteria),which enables appropriate cross-trial comparisons.Taken together,the data indicate that beyond the established role of brentuximab vedotin,CD30-directed CAR-T cells and bispecific antibodies demonstrate high activity in refractory cHL,especially when used with fludarabine-containing lymphodepletion,combined with programmed cell death 1(PD-1)receptor blockade as a strategy to eradicate minimal residual disease.Key challenges include durable effector-cell persistence and optimization of sequencing and combinations;notably,loss of CD30 as an escape mechanism appears uncommon.Integrating mechanistic insights into HRS biology with clinical trial data highlights strategies to enhance the efficacy,safety,and accessibility of CD30-directed immunotherapy.This review aims to provide a concise overview of CD30-targeted approaches in cHL,emphasizing therapeutic outcomes and the evolution of CAR-T technologies.
基金Supported by the Faculty of Medicine,Prince of Songkla University.Wainipitapong S has received grants from the Faculty of Medicine,Prince of Songkla University。
文摘AIM:To investigate the clinical characteristics and treatment outcomes,including visual function and overall survival(OS)of patients with ocular adnexal diffuse large B-cell lymphoma(OA-DLBCL).METHODS:This retrospective cohort study enrolled 29 patients diagnosed with OA-DLBCL based on histopathological biopsy between 2006 and 2023.Patients were stratified into two subgroups:primary OA-DLBCL(no prior history of lymphoma)and secondary OA-DLBCL(history of DLBCL at non-ocular adnexal sites).OS was defined as the time interval from OA-DLBCL diagnosis to death from any cause.Survival analysis was performed using the Kaplan–Meier method,and prognostic factors affecting OS were identified using multivariate Cox proportional hazards regression with a stepwise selection approach.RESULTS:The cohort included 24 patients with primary OA-DLBCL(13 males,11 females;mean age:61.36±18.29y)and 5 patients with secondary OA-DLBCL(2 males,3 females;mean age:50.94±18.17y).Among the primary OA-DLBCL subgroup,12 patients(50%)presented with advanced disease(Ann Arbor stage IIIE–IV),and 16 patients(66%)were classified as T4 disease according to the tumor-node-metastasis(TNM)staging system.The mean final visual acuity was 1.72±1.10 in the primary group and 0.90±1.18 in the secondary group.The 5-year OS rate for the entire cohort was 27.7%.Multivariate analysis identified five factors significantly associated with poor survival outcomes:epiphora[adjusted hazard ratio(aHR),36.95],atherosclerotic cardiovascular disease(aHR,10.08),human immunodeficiency virus(HIV)infection(aHR,12.47),M1 stage(aHR,6.99),and secondary OA-DLBCL(aHR,6.03;all P<0.05).The median OS was 1.68y for primary OA-DLBCL and 1.12y for secondary OA-DLBCL.CONCLUSION:A substantial proportion of patients with primary OA-DLBCL present with advanced-stage disease at diagnosis.Epiphora,atherosclerotic cardiovascular disease,HIV infection,M1 stage,and secondary OA-DLBCL are independent prognostic factors for poor survival outcomes.These findings emphasize the urgent need for optimized therapeutic strategies and early screening protocols to improve the management of OA-DLBCL,particularly in developing countries.
文摘Objective:We performed a population-based analysis focusing on primary extranodal lymphoma of either testis,kidney,bladder or prostate(PGUL).Methods:We identified all cases of localized testis,renal,bladder and prostate primary lymphomas(PL)versus primary testis,kidney,bladder and prostate cancers within the Surveillance,Epidemiology,and End Results database(1998e2015).Estimated annual proportion change methodology(EAPC),multivariable logistic regression models,cumulative incidence plots and multivariable competing risks regression models were used.Results:The rates of testis-PL,renal-PL,bladder-PL and prostate-PL were 3.04%,0.22%,0.18%and 0.01%,respectively.Patients with PGUL were older and more frequently Caucasian.Annual rates significantly decreased for renal-PL(EAPC:5.6%;pZ0.004)and prostate-PL(EAPC:3.6%;pZ0.03).In multivariable logistic regression models,older ager independently predicted testis-PL(odds ratio[OR]:16.4;p<0.001)and renal-PL(OR:3.5;p<0.001),while female gender independently predicted bladder-PL(OR:5.5;p<0.001).In surgically treated patients,cumulative incidence plots showed significantly higher 10-year cancer-specific mortality(CSM)rates for testis-PL,renal-PL and prostate-PL versus their primary genitourinary tumors.In multivariable competing risks regression models,only testis-PL(hazard ratio[HR]:16.7;p<0.001)and renal-PL(HR:2.52;p<0.001)independently predicted higher CSM rates.Conclusion:PGUL rates are extremely low and on the decrease in kidney and prostate but stable in testis and bladder.Relative to primary genitourinary tumors,PGUL are associated with worse CSM for testis-PL and renal-PL but not for bladder-PL and prostate-PL,even after adjustment for other-cause mortality.
基金Supported by the Department of Science and Technology of Sichuan Province,No.2023NSFSC0724.
文摘BACKGROUND Mantle cell lymphoma(MCL)is an aggressive B-cell non-Hodgkin lymphoma that rarely presents with cutaneous involvement,which typically occurs in advanced disease stages.Primary cutaneous manifestations are particularly uncommon and frequently misdiagnosed.We report a diagnostically challenging case of MCL that first appeared as a nodule on the lower leg.CASE SUMMARY An elderly female had a painless red nodule on the front of her right lower leg for six months.Positron emission tomography-computed tomography showed a soft tissue mass at the same site and increased activity in the right groin lymph nodes.Skin and lymph node biopsies,along with immunostaining,confirmed MCL.The patient began combination chemotherapy,resulting in a marked improvement of skin lesions and lymphadenopathy after two weeks.CONCLUSION Cutaneous manifestations,though uncommon,may serve as the initial clinical presentation of MCL.Dermatologists and pathologists should maintain heigh-tened awareness of this diagnostic possibility when evaluating persistent,atypical cutaneous nodules,particularly in elderly patients.Histopathological evaluation with complete immunophenotypic profiling is paramount for accurate diagnosis.
文摘BACKGROUND The concept of“duodenal-type follicular lymphoma(FL)”has already been established as a disease entity.On the other hand,because FL that develops in the colorectum is a rare disease,its clinical characteristics,including its treatment and outcome,are poorly understood.AIM To clarify the characteristic clinical features of colorectal FL.METHODS We identified 5 patients with FL from December 2010 to July 2022 whose colorectal lesions were confirmed by endoscopic biopsy at our hospital and retrospectively examined their clinical features,including endoscopic findings,treatment,and long-term outcomes.RESULTS The median age of the patients was 70 years(range 62-74 years,3 males and 2 females),and 2 patients were considered to have systemic FL lesions.Endoscopic findings revealed elavated lesions in all 5 patients(polypoid 3,flat elavated 1,papular 1).Only 1 patient underwent therapeutic intervention,and 4 patients chose watchful waiting.Except for 1 patient in which the lesion spontaneously regressed,the disease recurred or progressed in 4 patients,3 of whom eventually achieved near complete remission with the administration of rituximab and bendamustine(±polatuzumab vedotin).The 5 patients in this study were still alive or exhibited long-term survival before death;the survival time ranged from 8-29 years after the onset of lymphoma.CONCLUSION In the present study,colorectal FL progressed slowly,and overall survival was good because it was sensitive to anticancer drugs even after the disease progressed without initial therapeutic intervention.
文摘BACKGROUND Primary cardiac lymphoma(PCL)is a rare subset of cardiac tumors,often diagnosed late due to nonspecific symptoms.It predominantly affects immunocompromised individuals,primarily in the pericardium and right heart.Late diagnosis mimics common cardiac ailments,leading to poor prognosis.AIM To systematically review the efficacy of rituximab in treating PCL either alone or in various chemotherapeutic regimens.Secondary objectives include evaluating morphological subtypes,assessing treatment regimens,and analyzing outcomes focusing on remission and adverse events.METHODS Following PRISMA guidelines,a comprehensive literature search was conducted across multiple databases,including PubMed,Hinari,Web of Science,and Scopus.English-language studies reporting the use of rituximab in treating PCL in humans were included.Study selection involved initial screening of titles and abstracts followed by full-text examination and data extraction.RESULTS Thirty-three case reports involving 36 patients were included in this systematic review.Diffuse large B-cell lymphoma was the predominant morphological subtype observed.The rituximab,cyclophosphamide,doxorubicin,oncovin,and prednisolone regimen emerged as the most commonly employed treatment strategy,indicating widespread acceptance and efficacy in PCL management.Combination therapies,including surgical intervention,showed promise in achieving complete remission,while some studies reported mortality despite aggressive treatment approaches.CONCLUSION Rituximab,particularly in combination with chemotherapy regimens,represents a significant advancement in PCL management,offering hope for improved patient outcomes.However,challenges such as variable treatment responses and adverse events underscore the complexity of managing PCL.Further research is warranted to refine therapeutic strategies and enhance diagnostic approaches for this rare cardiac malignancy.
文摘BACKGROUND T/histiocyte-rich large B-cell lymphoma(T/HRBCL)is a highly aggressive subtype of diffuse large B-cell lymphoma characterized histologically by the presence of a few neoplastic large B cells amidst an abundant background of reactive T lymphocytes and/or histiocytes.T/HRBCL commonly affects the lymph nodes,followed by extranodal sites,such as the spleen,liver,and bone marrow,with rare occurrences in the gastrointestinal tract.Primary gastrointestinal T/HRBCL lacks specific clinical and endoscopic manifestations,and it is difficult to differentiate from inflammatory diseases,nodular lymphocyte predominant Hodgkin lymphoma,and other diseases on a histological basis,thereby hindering early diagnosis.CASE SUMMARY A 63-year-old man was hospitalized with a one-month history of jaundice and weight loss of approximately 3 kg.Laboratory tests revealed increased hepatic parameters in a cholestatic pattern and elevated carbohydrate antigen 19-9 levels.An abdominal computed tomography scan revealed a low-density mass within the descending duodenum and dilation of the bile and pancreatic ducts.He was clinically diagnosed with a duodenal tumor.During surgery,a 7.0 cm×8.0 cm mass was identified within the descending duodenum,so pancreaticoduodenectomy and cholecystectomy were performed.Following operative biopsy,the tumor was diagnosed as primary duodenal T/HRBCL.The patient refused postoperative chemotherapy and died four months after surgery.CONCLUSION Primary duodenal T/HRBCL is an extremely rare and highly aggressive malignancy.The initial treatment strategies should be based on the original site of the tumor,the disease stage,and the patient's physical condition.Chemotherapy-based comprehensive treatment is still the main treatment method for primary gastrointestinal T/HRBCL.
基金supported by grants from the Jinling Hospital Affiliated to Medical School of Nanjing University(2023LCZLXB055).
文摘Background:Primary bone marrow diffuse large B-cell lymphoma(PBM-DLBCL)represents an uncommon yet clinically aggressive hematologic malignancy.Despite its significant clinical impact,this entity lacks standardized diagnostic criteria in current WHO classifications.Methods:We performed a retrospective analysis of 55 PBM-DLBCL cases from our institutional database and published literature(2001–2022)to characterize disease features and identify prognostic factors,with particular focus on assessing how different treatment regimens influence therapeutic efficacy and long-term outcomes.Results:The data suggested a potential link between international prognostic index(IPI)scores and poorer survival,albeit without conclusive statistical evidence(p 0.05).Treatment=response emerged as a significant prognostic factor,and patients with complete response(CR)demonstrating superior survival in Cox univariate and multivariate analysis(p 0.001).Intensive therapeutic regimens were associated with<improved clinical outcomes compared to conventional therapies.While incorporating rituximab into conventional chemotherapy regimens has demonstrated superior clinical outcomes compared to chemotherapy alone in PBM-DLBCL patients.Conclusion:Our findings highlight the aggressive nature of PBM-DLBCL and underscore the importance of early recognition,risk stratification,and optimized treatment selection for this rare disease entity.
基金supported by the Sponsored by Tianjin Health Research Project(Grant No.TJWJ2023ZD003)the Chinese Society of Clinical Oncology Beijing Xisike Clinical Oncology Research Foundation(Grant Nos.Y-NCJH202201-0027 and Y-2022YMJN/MS-0001).
文摘Because human immunodeficiency virus(HIV)-associated Burkitt lymphoma(BL)has a poor prognosis new therapeutic approaches need to be developed1.Axicabtagene ciloleucel(axi-cel)is an anti-CD19 CAR-T cell commercially available FDA-approved product for patients with relapsed or refractory(R/R)large B-cell lymphoma(LBCL).However,axi-cel has not been approved by the FDA for use in patients with R/R BL.
文摘BACKGROUND Mucosa-associated lymphoid tissue(MALT)lymphoma is a subtype of extranodal marginal zone lymphoma,typically occurring in mucosal sites such as the stomach,salivary glands,and lungs.This study aims to analyze the demographic and clinicopathologic characteristics of patients with gastric MALT lymphoma in the United States and evaluate the interaction between age and gender on survival outcomes.AIM To analyze the demographic and clinicopathologic characteristics of patients with gastric MALT lymphoma in the United States and evaluate the interaction between age and gender on survival outcomes.METHODS A retrospective cohort study was conducted using the Surveillance,Epidemiology,and End Results(SEER)database,which included 2453 patients diagnosed with MALT lymphoma from 2010 to 2021.Data were analyzed for demographic factors,tumor characteristics,treatment modalities,and survival outcomes.A Cox proportional hazards regression model was used to identify predictors of overall mortality and cancer-specific mortality.RESULTS The study predominantly included Non-Hispanic White patients(62.78%),with nearly equal gender distribution(50.31%females,49.69%males),and most diagnoses occurring in individuals aged 60-79 years.The majority of tumors were localized(80.07%).Multivariate analysis identified older age,male gender,advanced tumor stage,and socioeconomic factors—such as annual income and marital status—as independent predictors of mortality.No significant interaction between age and gender on mortality outcomes was observed.CONCLUSION Sociodemographic factors,including advanced age,male gender,annual income,and marital status,as well as advanced tumor stage,significantly impacted survival outcomes in patients with MALT lymphoma.Radiotherapy was associated with a reduction in overall mortality.Early detection is crucial for optimizing outcomes,as localized disease responds well to available treatment modalities.
文摘BACKGROUND Primary testicular lymphoma(PTL)is a rare,aggressive malignancy,representing a small fraction of testicular tumors and non-Hodgkin lymphomas,yet it is the most common testicular malignancy in older men.Diffuse large B-cell lymphoma(DLBCL),which is typically the aggressive subtype,dominates PTL and shows diffuse B-cell infiltration.Venous tumor thrombus,uncommon in lymphomas,is uniquely reported in this case of testicular DLBCL with gonadal vein involvement.CASE SUMMARY A 62-year-old man presented with a two-month history of painless left testicular swelling and stiffness.Diagnostic imaging[ultrasonography,computed tomography(CT),and 18F-fluorodeoxyglucose positron emission tomography/CT(18FFDG-PET/CT)]revealed bilateral testicular masses and a gonadal vein tumor thrombus(SUVmax 16.5).Left orchiectomy confirmed DLBCL with CD20,Bcl-2,and MUM1 positivity(Ki-67:approximately 80%).The disease was staged as Ann Arbor stage IVA(International Prognostic Index score 3,high-intermediate risk).The patient received Rituximab,Polatuzumab Vedotin,Cyclophosphamide,Epirubicin,and Prednisolone chemotherapy,completing the first cycle with good tolerability.No adverse events were reported,and follow-up is ongoing to assess long-term outcomes.This case highlights the diagnostic utility of 18F-FDGPET/CT and the importance of multidisciplinary management in rare PTL presentations with tumor thrombus.CONCLUSION This case demonstrates the diagnostic complexities of PTL with gonadal vein tumor thrombus,underscoring the importance of considering lymphoma in elderly patients with testicular masses and venous involvement.A multi-disciplinary team including urologists,hematologists,and radiation oncologists is needed to ensure appropriate therapy.
文摘BACKGROUND In addition to nodal lesions,over 30%of mantle cell lymphomas(MCLs)also have gastrointestinal involvement,characteristically presenting as multiple lymphomatous polyposis(MLP),which rarely involve the esophagus.Most related papers have been case reports,and no comprehensive studies have been conducted;thus,the actual clinical situation has remained unknown for a long time.AIM To elucidate the actual clinical situation of esophageal involvement of MCL presenting with MLP,including its prognosis.METHODS From January 2001 to December 2021,among MCL patients whose gastrointestinal lesions were histopathologically confirmed by endoscopic biopsy at our center,6 patients with MLP in the esophagus were selected.We retro-spectively examined the clinical features of these patients,including their prognosis.RESULTS In all patients,multiple lesions were present in the gastrointestinal tract other than the esophagus and in the lymph nodes throughout the body,and most patients also had lesions involving the bone marrow or spleen.Most of the treatments include chemotherapy,with a 50%survival period of less than 2 years and a 5-year survival rate of approximately 30%,indicating a poor prognosis.CONCLUSION Patients with esophageal involvement of the MCL who presented with MLP had a large tumor burden and poor survival.
基金supported by funding from the Veterans Administration(I01 BX006101-01)supported in part by funding from the Veterans Administration(IO1 BX001262)the recipient of RCS Award(IK6 BX005964)from the Department of Veterans Administration.
文摘Fc Receptor-Like 1(FCRL1),a member of the FCRL family,contains two immunoreceptor tyrosinebased activation motifs(ITAMs)in its cytoplasmic domain and plays a critical role in B-cell biology.Its expression begins in pre-B-cells,dynamically shifts during B-cell development,and contributes to the regulation of human B-cell activation.Notably,FCRL1 is overexpressed in subsets of naive and memory B-cells,as well as in malignant B-cells,including those in diffuse large B-cell lymphoma(DLBCL),an aggressive and often treatment-resistant hematological malignancy.Among FCRL family members,FCRL1 stands out as a promising immunotherapeutic target due to its selective expression in malignant B-cells and its functional role in proliferation.Given the limited efficacy of current therapies for relapsed/refractory DLBCL,targeting FCRL1 could address an unmet clinical need by offering a novel,mechanism-based approach to modulate B-cell signaling and enhance anti-tumor immunity.This mini-review highlights the therapeutic potential of FCRL1-directed strategies,supporting their further exploration in preclinical models and future clinical trials for DLBCL and other B-cell malignancies.
文摘AIM: To evaluate the endoscopic manifestations and prognoses of gastrointestinal (GI) mantle cell lymphoma (MCL). METHODS: A database search at the Department of Pathology of Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences revealed 57 MCL patients with GI involvement. Clinical records were available for 35 of the 57 patients from 21 institutions, and those 35 patients were enrolled in this study. We summarized the gross types of endoscopic features, event-free survival (EFS), and overall survival (OS) of those patients.RESULTS: Of the 35 patients, GI involvement in the esophagus, stomach, and duodenum was found in 2 (5.7%), 26 (74.3%), and 12 (34.3%) patients, respectively. Twenty-one of the 35 patients underwent colonoscopy; among them, GI involvement in the ileum, cecum, colon, and rectum was found in 10 (47.6%), 3 (14.3%), 12 (57.1%), and 10 (47.6%), respectively. Various lesions, such as superficial, protruded, fold thickening, or ulcerative, were found in the stomach, whereas multiple lymphomatous polyposis (MLP) was dominant from the duodenum to the rectum. Twelve patients were treated with a hyper-CVAD/MA regimen, and they had better OS (3-year rate, 88.3% vs 46.4%, P < 0.01) and better EFS (3-year rate, 66.7% vs 33.8%, P < 0.05) than the remaining 23 patients who were not treated with this regimen. CONCLUSION: MLP was a representative form of intestinal involvement, whereas a variety of lesions were found in the stomach. The hyper-CVAD/MA regimen may improve survival in these patients.
文摘BACKGROUND Primary central nervous system lymphoma(PCNSL)is a non-Hodgkin lymphoma that originates in the central nervous system(CNS)and is exclusively limited to the CNS.Although most PCNSLs are diffuse large B-cell lymphomas,primary CNS T-cell lymphomas(PCNSTLs)are rare.PCNSTLs typically demonstrate some degree of enhancement on contrast-enhanced magnetic resonance imaging(MRI).To the best of our knowledge,non-enhancing PCNSTL has not been reported previously.CASE SUMMARY A 69-year-old male presented to the neurology department with complaints of mild cognitive impairment and gradual onset of left lower leg weakness over a span of two weeks.Initial MRI showed asymmetric T2-hyperintense lesions within the brain.No enhancement was observed on the contrast-enhanced T1 image.The initial diagnosis was neuro-Behçet’s disease.Despite high-dose steroid therapy,no alterations in the lesions were identified on initial MRI.The patient’s symptoms deteriorated further.An MRI performed one month after the initial scan revealed an increased lesion extent.Subsequently,brain biopsy confirmed the diagnosis of PCNSTL.The patient underwent definitive combined chemoradiotherapy.However,the patient developed bacteremia and died of septic shock approximately three months after diagnosis.CONCLUSION The absence of enhancement in the lesion did not rule out PCNSTL.A biopsy approach is advisable for pathological confirmation.
基金Supported by the Outstanding Young Talents Program of Air Force Medical Center,People’s Liberation Army,No.22BJQN004Clinical Program of Air Force Medical University,No.Xiaoke2022-07.
文摘BACKGROUND Primary gastrointestinal lymphoma(PGIL)is a relatively uncommon clinical entity,exhibiting distinctive features including occult primary sites,nonspecific clinical presentations,and considerable diagnostic and therapeutic difficulties.Consequently,comprehensive clinical investigations into its clinicopathological characteristics and surgical intervention value are warranted to enhance dia-gnostic and therapeutic proficiency.AIM To investigate the clinicopathological characteristics and surgical significance of PGIL from a surgical perspective,providing a theoretical basis for optimizing diagnostic and therapeutic strategies.METHODS This study included 50 cases of PGIL treated by the General Surgery Department of the Chinese PLA Air Force Medical Center from June 2001 to March 2025.Data were extracted from the Electronic Medical Record system for retrospective analysis.A retrospective analysis was conducted on their epidemiological,clinical manifestations,imaging,pathological features,and treatment outcomes.Descriptive statistics were applied for data summarization,with continuous variables presented as frequencies and percentages.Correlations between variables were assessed using the Spearman rank correlation coefficient.RESULTS All cases had the gastrointestinal tract as the primary site.Abdominal pain was the most common initial symptom(52.0%),with 80.0%of patients experiencing pain during the course of the disease,and 38.0%experiencing hema-tochezia/melena or anemia.Computed tomography diagnosis exhibited a high overall sensitivity(94.3%);the en-doscopic detection rate was 91.5%.Diffuse large B-cell lymphoma was the most common subtype(52.0%).The im-provement rate was higher in the surgery combined with chemotherapy group than in the chemotherapy only group.The incidence of postoperative complications was 26.5%,all occurring in patients with tumors>5 cm.CONCLUSION Diffuse large B-cell lymphoma is the primary PGIL subtype.Imaging and endoscopic biopsy are diagnostic es-sentials.Surgery aids in resection,complication management,and pathologic diagnosis.Multidisciplinary,indi-vidualized strategies are recommended,necessitating further prospective molecular studies.
基金Supported by the National Natural Science Foundation of China,No.82160330the Science and Technology Program of the Health Commission of Jiangxi Province,No.2025110045+1 种基金the Ganzhou Science and Technology Planning Project,No.GZ2024YLJ016,No.GZ2024YLJ026,and No.GZ2024ZSF064the Ganzhou Health Commission Scientific Research Planning Project,No.GZWJW202402108.
文摘BACKGROUND Monomorphic epitheliotropic intestinal T-cell lymphoma(MEITL)is an uncom-mon and highly aggressive form of lymphoma that represents less than 1%of all non-Hodgkin’s lymphomas.At present,few reports have focused on the imaging findings of MEITL,which poses significant challenges for clinical diagnosis.A 78-year-old female with recurrent vomiting and abdominal distension was admitted to our hospital.Magnetic resonance and^(18)F-fluorodeoxyglucose positron emission tomography/computed tomography(^(18)F-FDG PET/CT)examinations revealed multiple segmental malignant tumors in the small intestine with me-senteric lymph node metastasis.An endoscopic biopsy revealed MEITL.After three cycles of reduced-dose cyclophosphamide,vinorelbine,and prednisone che-motherapy,follow-up^(18)F-FDG PET/CT demonstrated a partial response to treatment.The patient was still alive after 6 months of follow-up.CONCLUSION Magnetic resonance imaging serves as a valuable tool in detecting malignant tumor lesions of MEITL,whereas^(18)F-FDG PET/CT offers additional assistance in tumor staging and assessing treatment efficacy.
