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1.
J Hematop ; 2024 Jul 20.
Artigo em Inglês | MEDLINE | ID: mdl-39031273

RESUMO

Mantle cell lymphoma (MCL) is a rare and aggressive type of lymphoma that can affect the kidneys. The disease can lead to kidney impairment, and glomerulonephritis (GN) is a rare but serious complication of MCL. We report a case of MCL with kidney interstitial infiltration and membranoproliferative glomerulonephritis with focal and segmental glomerulosclerosis. A 75-year-old man presented recurrent acute kidney failure and worsening of nephrotic syndrome. Kidney biopsy revealed membranoproliferative glomerulonephritis presented immunoglobulin and complement deposition, focal and segmental glomerulosclerosis of not otherwise specified type, and infiltration by mantle cell lymphoma. Bone marrow biopsy and PET/CT scan confirmed the diagnosis of mantle cell lymphoma. The patient was treated with R-CHOP21 chemotherapy with cyclophosphamide dose adjustment for nephroprotection. He achieved complete remission with normalization of hematological parameters, improvement of kidney function, and reduction of proteinuria and albuminuria. This case shows the importance of considering alternative diagnoses in patients with recurrent chronic kidney disease and worsening nephrotic syndrome. Early diagnosis and treatment of mantle cell lymphoma can lead to favorable outcomes.

2.
Leuk Lymphoma ; : 1-14, 2024 Jul 08.
Artigo em Inglês | MEDLINE | ID: mdl-38975903

RESUMO

To quantify the clinical unmet need of r/r MCL patients who progress on a covalent Bruton tyrosine kinase inhibitor (BTKi), we conducted a systematic review to identify studies that reported overall survival (OS), progression-free survival (PFS), or response outcomes of patients who received a chemo(immunotherapy) ± targeted agent standard therapy (STx) or brexucabtagene autoleucel (brexu-cel) in the post-BTKi setting. Twenty-six studies (23 observational; three trials) reporting outcomes from 2005 to 2022 were included. Using two-stage frequentist meta-analyses, the estimated median PFS/OS for patients treated with an STx was 7.6 months (95% CI: 3.9-14.6) and 9.1 months (95% CI: 7.3-11.3), respectively. The estimated objective response rate (ORR) was 45% (95% CI: 34-57%). For patients treated with brexu-cel, the estimated median PFS/OS was 14.9 months (95% CI: 10.5-21.0) and 32.1 months (95% CI: 25.2-41.2), with a pooled ORR of 89% (95% CI: 86-91%). Our findings highlight a significant unmet need for patients whose disease progresses on a covalent BTKi.

3.
Acta Pharm Sin B ; 14(7): 2977-2991, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-39027231

RESUMO

Cyclin D1 has been recognized as an oncogene due to its abnormal upregulation in different types of cancers. Here, we demonstrated that cyclin D1 is SUMOylated, and we identified Itch as a specific E3 ligase recognizing SUMOylated cyclin D1 and mediating SUMO-induced ubiquitination and proteasome degradation of cyclin D1. We generated cyclin D1 mutant mice with mutations in the SUMOylation site, phosphorylation site, or both sites of cyclin D1, and found that double mutant mice developed a Mantle cell lymphoma (MCL)-like phenotype. We showed that arsenic trioxide (ATO) enhances cyclin D1 SUMOylation-mediated degradation through inhibition of cyclin D1 deSUMOylation enzymes, leading to MCL cell apoptosis. Treatment of severe combined immunodeficiency (SCID) mice grafted with MCL cells with ATO resulted in a significant reduction in tumor growth. In this study, we provide novel insights into the mechanisms of MCL tumor development and cyclin D1 regulation and discover a new strategy for MCL treatment.

4.
Int J Mol Sci ; 25(13)2024 Jul 02.
Artigo em Inglês | MEDLINE | ID: mdl-39000404

RESUMO

Mantle cell lymphoma (MCL) is a rare, incurable, and aggressive B-cell non-Hodgkin lymphoma (NHL). Early MCL diagnosis and treatment is critical and puzzling due to inter/intra-tumoral heterogeneity and limited understanding of the underlying molecular mechanisms. We developed and applied a multifaceted analysis of selected publicly available transcriptomic data of well-defined MCL stages, integrating network-based methods for pathway enrichment analysis, co-expression module alignment, drug repurposing, and prediction of effective drug combinations. We demonstrate the "butterfly effect" emerging from a small set of initially differentially expressed genes, rapidly expanding into numerous deregulated cellular processes, signaling pathways, and core machineries as MCL becomes aggressive. We explore pathogenicity-related signaling circuits by detecting common co-expression modules in MCL stages, pointing out, among others, the role of VEGFA and SPARC proteins in MCL progression and recommend further study of precise drug combinations. Our findings highlight the benefit that can be leveraged by such an approach for better understanding pathobiology and identifying high-priority novel diagnostic and prognostic biomarkers, drug targets, and efficacious combination therapies against MCL that should be further validated for their clinical impact.


Assuntos
Reposicionamento de Medicamentos , Linfoma de Célula do Manto , Linfoma de Célula do Manto/diagnóstico , Linfoma de Célula do Manto/tratamento farmacológico , Linfoma de Célula do Manto/genética , Linfoma de Célula do Manto/metabolismo , Linfoma de Célula do Manto/patologia , Humanos , Reposicionamento de Medicamentos/métodos , Regulação Neoplásica da Expressão Gênica/efeitos dos fármacos , Redes Reguladoras de Genes/efeitos dos fármacos , Osteonectina/metabolismo , Osteonectina/genética , Fator A de Crescimento do Endotélio Vascular/metabolismo , Fator A de Crescimento do Endotélio Vascular/genética , Transcriptoma , Perfilação da Expressão Gênica/métodos , Biomarcadores Tumorais/metabolismo , Transdução de Sinais/efeitos dos fármacos , Antineoplásicos/uso terapêutico , Antineoplásicos/farmacologia
5.
Cancers (Basel) ; 16(13)2024 Jun 21.
Artigo em Inglês | MEDLINE | ID: mdl-39001353

RESUMO

With the aim to advance the understanding of immune regulation in MCL and to identify targetable T-cell subsets, we set out to combine image analysis and spatial omic technology focused on both early and late differentiation stages of T cells. MCL patient tissue (n = 102) was explored using image analysis and GeoMx spatial omics profiling of 69 proteins and 1812 mRNAs. Tumor cells, T helper (TH) cells and cytotoxic (TC) cells of early (CD57-) and late (CD57+) differentiation stage were analyzed. An image analysis workflow was developed based on fine-tuned Cellpose models for cell segmentation and classification. TC and CD57+ subsets of T cells were enriched in tumor-rich compared to tumor-sparse regions. Tumor-sparse regions had a higher expression of several key immune suppressive proteins, tentatively controlling T-cell expansion in regions close to the tumor. We revealed that T cells in late differentiation stages (CD57+) are enriched among MCL infiltrating T cells and are predictive of an increased expression of immune suppressive markers. CD47, IDO1 and CTLA-4 were identified as potential targets for patients with T-cell-rich MCL TIME, while GITR might be a feasible target for MCL patients with sparse T-cell infiltration. In subgroups of patients with a high degree of CD57+ TC-cell infiltration, several immune checkpoint inhibitors, including TIGIT, PD-L1 and LAG3 were increased, emphasizing the immune-suppressive features of this highly differentiated T-cell subset not previously described in MCL.

6.
J Transl Med ; 22(1): 622, 2024 Jul 04.
Artigo em Inglês | MEDLINE | ID: mdl-38965536

RESUMO

BACKGROUND: Inhibition of kinases is the ever-expanding therapeutic approach to various types of cancer. Typically, assessment of the treatment response is accomplished by standard, volumetric imaging procedures, performed weeks to months after the onset of treatment, given the predominantly cytostatic nature of the kinase inhibitors, at least when used as single agents. Therefore, there is a great clinical need to develop new monitoring approaches to detect the response to kinase inhibition much more promptly. Noninvasive 1H magnetic resonance spectroscopy (MRS) can measure in vitro and in vivo concentration of key metabolites which may potentially serve as biomarkers of response to kinase inhibition. METHODS: We employed mantle cell lymphoma (MCL) cell lines demonstrating markedly diverse sensitivity of inhibition of Bruton's tyrosine kinase (BTK) regarding their growth and studied in-depth effects of the inhibition on various aspects of cell metabolism including metabolite synthesis using metabolomics, glucose and oxidative metabolism by Seahorse XF technology, and concentration of index metabolites lactate, alanine, total choline and taurine by 1H MRS. RESULTS: Effective BTK inhibition profoundly suppressed key cell metabolic pathways, foremost pyrimidine and purine synthesis, the citrate (TCA) cycle, glycolysis, and pyruvate and glutamine/alanine metabolism. It also inhibited glycolysis and amino acid-related oxidative metabolism. Finally, it profoundly and quickly decreased concentration of lactate (a product of mainly glycolysis) and alanine (an indicator of amino acid metabolism) and, less universally total choline both in vitro and in vivo, in the MCL xenotransplant model. The decrease correlated directly with the degree of inhibition of lymphoma cell expansion and tumor growth. CONCLUSIONS: Our results indicate that BTK inhibition exerts a broad and profound suppressive effect on cell metabolism and that the affected index metabolites such as lactate, alanine may serve as early, sensitive, and reliable biomarkers of inhibition in lymphoma patients detectable by noninvasive MRS-based imaging method. This kind of imaging-based detection may also be applicable to other kinase inhibitors, as well as diverse lymphoid and non-lymphoid malignancies.


Assuntos
Tirosina Quinase da Agamaglobulinemia , Linfoma de Célula do Manto , Inibidores de Proteínas Quinases , Humanos , Linhagem Celular Tumoral , Inibidores de Proteínas Quinases/farmacologia , Animais , Tirosina Quinase da Agamaglobulinemia/metabolismo , Tirosina Quinase da Agamaglobulinemia/antagonistas & inibidores , Linfoma de Célula do Manto/metabolismo , Linfoma de Célula do Manto/patologia , Linfoma de Célula do Manto/tratamento farmacológico , Transdução de Sinais/efeitos dos fármacos , Biomarcadores Tumorais/metabolismo , Proliferação de Células/efeitos dos fármacos , Ensaios Antitumorais Modelo de Xenoenxerto , Camundongos , Biomarcadores/metabolismo
7.
Sci Rep ; 14(1): 16946, 2024 Jul 23.
Artigo em Inglês | MEDLINE | ID: mdl-39043871

RESUMO

In the frontline high-dose phase 3 FIL-MCL0208 trial (NCT02354313), 8% of enrolled mantle cell lymphoma (MCL) patients could not be randomised to receive lenalidomide (LEN) maintenance vs observation after autologous stem cell transplantation (ASCT) due to inadequate hematological recovery and 52% of those who started LEN, needed a dose reduction due to toxicity. We therefore focused on the role played by CD34 + hematopoietic stem cells (PBSC) harvesting and reinfusion on toxicity and outcome. Overall, 90% (n = 245) of enrolled patients who underwent the first leukapheresis collected ≥ 4 × 106 PBSC/kg, 2.6% (n = 7) mobilized < 4 × 106 PBSC/kg and 7.7% (n = 21) failed the collection. Similar results were obtained for the planned second leukapheresis, with only one patient failing both attempts. Median count of reinfused PBSC was 5 × 106/kg and median time to recovery from neutropenia G4 was 10 days from ASCT. No impact of mobilizing subtype or number of reinfused PBSC on hematological recovery and LEN dose reduction was noted. At a median follow-up of 75 months from ASCT, PFS and OS of transplanted patients were 50% and 73%, respectively. A long lasting G4 neutropenia after ASCT (> 10 days) was associated with a worse outcome, both in terms of PFS and OS. In conclusion, although the harvesting procedures proved feasible for younger MCL patients, long-lasting cytopenia following ASCT remains a significant issue: this can hinder the administration of effective maintenance therapies, potentially increasing the relapse rate and negatively affecting survival outcomes.


Assuntos
Mobilização de Células-Tronco Hematopoéticas , Leucaférese , Linfoma de Célula do Manto , Transplante Autólogo , Humanos , Linfoma de Célula do Manto/terapia , Pessoa de Meia-Idade , Masculino , Feminino , Mobilização de Células-Tronco Hematopoéticas/métodos , Leucaférese/métodos , Idoso , Adulto , Transplante de Células-Tronco Hematopoéticas/métodos , Lenalidomida/administração & dosagem , Lenalidomida/uso terapêutico , Células-Tronco Hematopoéticas/metabolismo , Antígenos CD34/metabolismo , Itália
8.
Int J Surg Case Rep ; 121: 109963, 2024 Jun 27.
Artigo em Inglês | MEDLINE | ID: mdl-38954973

RESUMO

INTRODUCTION AND IMPORTANCE: Mantle cell lymphoma is a rare type of non-Hodgkin's lymphoma which accounts for 5 % of all cases. Patients present with an advanced form of the disease. We present here a case of ileocolic intussusception secondary to mantle cell lymphoma which was revealed by abdominal pain and vomiting that was treated by surgical resection followed by chemotherapy. CASE PRESENTATION: This report illustrates the case of a 34-year-old male who presented with abdominal pain and vomiting. Imageology demonstrated an ileocolic intussusception which was treated with hemicolectomy followed by chemotherapy. Histopathology confirmed the diagnosis of Mantle cell lymphoma. CLINICAL DISCUSSION: Mantel cell lymphoma is a rare type of B-cell cancer. Patients are generally diagnosed with an advanced stage of the disease. Ileocolic intussusception is an uncommon presentation. Surgery is the pillar of the treatment. Resection depends on the extent and location of the lesion. Postoperative chemotherapy is crucial and it increases survival rate. CONCLUSION: Mantle cell lymphoma is a rare subgroup of B-cell lymphomas. Ileocolic intussusception is a complicated form of the disease. Surgery combined with chemotherapy is the mainstay of the treatment. Diagnosis is confirmed by histological analysis of the surgical specimen.

9.
Am J Transl Res ; 16(6): 2599-2606, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-39006292

RESUMO

Composite lymphoma (CL) is a rare cancer characterized by the concurrent occurrence of more than one type of lymphoma within the same organ or tissue in an individual. Its occurrence at extranodal sites is exceptional, with only a few cases documented in the literature. A 62-year-old gentleman presented with dystonia, dysphagia, and irregular enlargement of the right tonsil for the last three months. Based on a clinical suspicion of tonsillar malignancy, tonsillectomy was done. The histopathologic examination revealed effacement of the architecture by large irregular lymphoid nodules with interfollicular expansion. The nodules showed sheets of small atypical lymphoid cells, while the interfollicular areas showed large atypical lymphoid cells with scattered typical binucleate Reed-Sternberg cells. Immunohistochemistry confirmed mantle cell lymphoma (MCL) in the nodules and classical HL (cHL) in the interfollicular areas. Based on these features, a diagnosis of composite MCL with cHL was rendered. He was treated with bendamustine and rituximab chemotherapy and remained in complete remission for five years when he presented with significant right-sided neck swelling. Percutaneous fine needle aspiration and subsequent flow cytometry confirmed a relapse of the MCL component of the CL. The index report documents an exceptional case of CL, comprising MCL and cHL, presenting at an uncommon extranodal site. In addition, it also emphasizes the importance of adequate sampling and the simultaneous use of immunochemistry and/or flow cytometry to confirm the presence of more than a single type of lymphoma, which may be easily overlooked on microscopy alone.

10.
Front Oncol ; 14: 1394057, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-39011476

RESUMO

Chimeric antigen receptor (CAR) T-cell therapy is a revolutionary approach in the treatment of lymphoma. This review article provides an overview of the four FDA-approved CAR T-cell products for aggressive B-cell lymphoma, including diffuse large B-cell lymphoma and mantle cell lymphoma, highlighting their efficacy and toxicity as well as discussing future directions.

11.
Artigo em Inglês | MEDLINE | ID: mdl-39034204

RESUMO

BACKGROUND: While covalent Bruton's tyrosine kinase inhibitors (cBTKis) have become a standard of care treatment for relapsed/refractory mantle cell lymphoma (R/R MCL), response duration is limited and resistance to BTKi and/or adverse events develop in a subset of patients. However, little real-world evidence on post-cBTKi clinical and economic outcomes exists for these patients. PATIENTS AND METHODS: This retrospective study used 2010 to 2019 U.S. Medicare claims, to identify elderly (≥ 66 years) patients with newly-diagnosed MCL who received third-line (3L) treatment and had evidence of cBTKi use in a prior line of therapy. Outcomes were assessed ≥ 12-months post 3L-treatment initiation and included treatment patterns, all-cause and MCL-related HRU and costs, and overall survival. RESULTS: The final sample contained 230 elderly patients with R/R MCL receiving 3L treatment who had cBTKi use in a prior line of therapy (mean age 75.0, 21.7% age > 80 years; 67.4% male; 93.9% White). Common 3L treatments included chemotherapy (26.1%), lenalidomide (18.7%), and bortezomib (18.3%); 1-quarter (25.7%) of patients received a cBTKi (17.8% ibrutinib; 7.8% acalabrutinib). Overall survival was poor from 3L treatment initiation (median OS = 9.4 months; 1-years survival rate = 43.7%). Patients exhibited high rates of HRU (73.6% experienced hospitalization) and substantial costs ($145,726) in the 12-months after 3L initiation. CONCLUSION: A large unmet need exists in this patient subpopulation, highlighting the importance of ongoing development of novel therapeutics.

12.
BMC Nephrol ; 25(1): 225, 2024 Jul 15.
Artigo em Inglês | MEDLINE | ID: mdl-39009965

RESUMO

BACKGROUND: Membranous nephropathy (MN) is a common type of nephrotic syndrome (NS) in adults, accounting for about 20-30% of cases. Although secondary to specific factors, the coexistence of MN and mantle cell lymphoma (MCL) has been scarcely reported in clinical literature. CASE PRESENTATION: A 59-year-old Chinese male was admitted to the hospital with a generalized pruritic rash with bilateral lower extremity edema, which did not improve significantly after symptomatic treatment. He had undergone renal biopsy, and the diagnosis was thought to be secondary MN (SMN), therefore, we did a lymph node biopsy on the patient and found that MN was complicated with MCL. Soon after, the patient was admitted to the hematology department for a BR chemotherapy regimen (composed of bendamustine 90 mg/m2 BSA (body surface area), rituximab 375 mg/m2 BSA and dexamethasone 5 mg), and during the post-treatment follow-up, both his symptoms and renal function improved. CONCLUSIONS: The mechanism underlying the combination of SMN and MCL remains elusive and exceedingly rare, consequently often overlooked in clinical practice. This case serves to offer valuable clinical insights for diagnosis and treatment, while emphasizing the pivotal role of renal pathology in clinical assessment.


Assuntos
Exantema , Síndrome Nefrótica , Humanos , Masculino , Pessoa de Meia-Idade , Síndrome Nefrótica/complicações , Síndrome Nefrótica/diagnóstico , Síndrome Nefrótica/etiologia , Síndrome Nefrótica/tratamento farmacológico , Exantema/etiologia , Exantema/tratamento farmacológico , Linfoma de Célula do Manto/complicações , Linfoma de Célula do Manto/tratamento farmacológico , Linfoma de Célula do Manto/diagnóstico , Glomerulonefrite Membranosa/complicações , Glomerulonefrite Membranosa/diagnóstico , Glomerulonefrite Membranosa/tratamento farmacológico , Rituximab/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Dexametasona/uso terapêutico , Dexametasona/administração & dosagem , Cloridrato de Bendamustina/uso terapêutico , Cloridrato de Bendamustina/administração & dosagem
13.
Biologics ; 18: 181-193, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38979130

RESUMO

Objective: The purpose of this study was to analyze the mechanism by which STAT5B inhibits ferroptosis in mantle cell lymphoma (MCL) by promoting DCAF13 transcriptional regulation of p53/xCT pathway. Methods: The correlations between STAT5B, DCAF13 and ferroptosis in MCL were analyzed using Gene Expression Profiling Interactive Analysis (GEPIA, http://gepia.cancer-pku.cn/index.html). The expression levels and pairwise correlations of STAT5B, DCAF13, p53 and xCT in MCL patients were detected, respectively. STAT5B was silenced to confirm their criticality in MCL ferroptosis. the effects of blocking necrosis, apoptosis and ferroptosis on the anti-MCL effects of STAT5B were examined. Cells with STAT5B overexpression and/or DCAF13 silencing were constructed to confirm the involvement of DCAF13 in the STAT5B-regulated p53/xCT pathway. The regulation of p53 ubiquitination was confirmed by DCAF13 overexpression and MG132. The effects of silencing DCAF13 and MG132 on STAT5B overexpression on MCL was clarified by a tumor-bearing nude mouse model. Results: DCAF13 was overexpressed in MCL and positively correlated with STAT5B, negatively correlated with p53, and positively correlated with xCT. Inhibition of ferroptosis alleviated the inhibitory effects of siSTAT5B on MCL, while inhibition of necrosis and apoptosis had few effects. Silencing of DCAF13 led to the blocking of STAT5B regulation of p53/xCT and ferroptosis. The changes in DCAF13 and the addition of MG132 did not have statistically significant effects on p53 mRNA. Elevation of DCAF13 resulted in downregulation of p53 protein levels, and this inhibition was reversed by MG132. In animal models, the promotion of MCL and the inhibition of ferroptosis by STAT5B. Silencing of DCAF13 blocked STAT5B inhibition of p53 and induction of xCT, GPX4, and GSH. Conclusion: STAT5B suppresses ferroptosis by promoting DCAF13 transcription to regulate p53/xCT pathway to promote MCL progression.

14.
Zhongguo Yi Xue Ke Xue Yuan Xue Bao ; 46(3): 458-461, 2024 Jun.
Artigo em Chinês | MEDLINE | ID: mdl-38953271

RESUMO

Intestinal mantle cell lymphoma complicated with intussusception is rare in clinical practice,lacking specific clinical manifestations.CT and colonoscopy are helpful for the diagnosis of this disease,which need to be distinguished from colorectal cancer,Crohn's disease,and other pathological subtypes of lymphoma.The diagnosis still needs to be confirmed by pathological examination.This paper reports a case of intestinal mantle cell lymphoma complicated with ileocecal intussusception in an adult,aiming to improve the clinical and imaging doctors' understanding of this disease.


Assuntos
Doenças do Íleo , Intussuscepção , Linfoma de Célula do Manto , Humanos , Linfoma de Célula do Manto/complicações , Intussuscepção/etiologia , Intussuscepção/diagnóstico por imagem , Intussuscepção/complicações , Masculino , Doenças do Íleo/etiologia , Doenças do Íleo/complicações , Doenças do Íleo/diagnóstico por imagem , Neoplasias Intestinais/complicações , Neoplasias Intestinais/patologia , Neoplasias Intestinais/diagnóstico por imagem , Pessoa de Meia-Idade , Valva Ileocecal/diagnóstico por imagem , Valva Ileocecal/patologia
15.
Pan Afr Med J ; 47: 111, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38828423

RESUMO

Mantle cell lymphoma (MCL) accounts for 3-10% of non-Hodgkin's lymphomas (NHL). We identified 14 patients with mantle cell lymphoma, with an average number of 3.5 new cases/year. A male predominance was observed with a sex ratio equal to 6. The average age of our patients was 64.4±14.1 years, with an average diagnostic delay of 6.57 months. Regarding the clinical presentation, adenopathy was the most reported physical sign (78.6%) followed by B symptoms (57.1%). Disseminated stages were the most frequent in our series: stages IV (78.5%) and III (7.1%) versus stages I (0%) and II (7.1%). The extra-ganglionic localizations observed were hepatic 5 cases (31.1%), pulmonary 04 cases (25%), medullary 4 cases (25%), pleural 2 cases (12.5%) and prostate 1 case (6.2%). All diagnosed cases are mantle cell lymphomas, of which 12 cases (85.7%) are classical and 2 cases (14.3%) indolent. The high-risk group is, according to international prognostic index (MIPI) MCL prognostic score, the most represented in our series: 0-3 = 6 cases (42.9%), 6-11 = 8 cases (57.1%). The therapeutic protocol chosen 1st line: 9 patients treated with R-DHAP, three with R-CHOP, one with DHAOX and one with R-CVP. Second line: two patients treated with R-DHAP, one after R-CHOP and the other after R-CVP. Two patients received autologous hematopoietic stem cell transplant at the end of the treatment. The evolution was marked by the death of 7 patients, 3 lost to follow-up and 4 still followed. Additionally, the study highlights characteristics and treatment patterns of mantle cell lymphoma, emphasizing its predominance in males, delayed diagnosis, frequent dissemination, and high-risk classification, with chemotherapy as the primary treatment modality and a challenging prognosis contributing to a comprehensive understanding of mantle cell lymphoma presentation and management.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica , Linfoma de Célula do Manto , Estadiamento de Neoplasias , Humanos , Linfoma de Célula do Manto/terapia , Linfoma de Célula do Manto/patologia , Linfoma de Célula do Manto/diagnóstico , Linfoma de Célula do Manto/epidemiologia , Linfoma de Célula do Manto/tratamento farmacológico , Marrocos , Masculino , Pessoa de Meia-Idade , Feminino , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Idoso de 80 Anos ou mais , Adulto , Prognóstico , Estudos Retrospectivos , Diagnóstico Tardio , Ciclofosfamida/administração & dosagem , Vincristina/administração & dosagem
16.
Clin Case Rep ; 12(6): e8887, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38831984

RESUMO

Key Clinical Message: Patients presenting with abdominal pain and retroperitoneal mass in radiographic images may be in the early stages of primary adrenal mantle cell lymphoma, which requires histological studies for a definite diagnosis. Abstract: This report presents a 37-year-old woman complaining of ambiguous abdominal pain, with imaging findings revealing a retroperitoneal abdominal mass on the left side of the aorta, and a possible diagnosis of non-functional retroperitoneal paraganglioma. Total laparoscopic excision was performed. Surprisingly, histological examinations revealed features in favor of mantle cell lymphoma.

17.
J Clin Aesthet Dermatol ; 17(5-6 Suppl 1): S16-S18, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38872927

RESUMO

Mantle cell lymphoma (MCL) can, in a few reported cases, create an exaggerated mosquito bite response, leading to numerous bullae and significant edema. The phenomenon is further complicated by the observance of a concurrent Epstein-Barr virus (EBV) reactivation. The current literature provides general information on treatment that most practitioners would consider when attempting to treat an arthropod hypersensitivity reaction, such as topical or systemic corticosteroids. However, no information has been published to date that details a preventive and steroid-sparing approach to treating this phenomenon, without simultaneously treating the MCL. MCL can be indolent in nature and does not always require prompt treatment. The purpose of this case report is to discuss the successful treatment of a rare disorder with a steroid-sparing regimen. The steroid-sparing regimen used consisted of oral doxycycline 100mg twice daily, cetirizine 20mg once daily, and valacyclovir 1g daily, which resulted in sustained reduction in bullous eruptions.

18.
Br J Haematol ; 2024 Jun 28.
Artigo em Inglês | MEDLINE | ID: mdl-38938122

RESUMO

Mantle cell lymphoma (MCL) is a rare lymphoproliferative neoplasm considered incurable, with a median survival of 3-5 years. In recent years, Bruton's tyrosine kinase inhibitors (BTKi) have been introduced, demonstrating high therapeutic activity. However, the prognosis for MCL patients failing ibrutinib therapy is particularly poor, with a survival expectation of a few months. In this phase II trial, we assessed the efficacy and safety of the carfilzomib-lenalidomide-dexamethasone (KRD) combination in MCL patients who were relapsed/refractory (R/R) or intolerant to BTKi and in need of treatment. The primary objective of the study was to evaluate the antitumor efficacy of the KRD combination in terms of 12-month overall survival (12-month OS). From September 2019 to December 2020, 16 patients were enrolled from 11 Italian centers. After a median follow-up of 2.37 months (95% CI 0.92-6.47), the 12-month OS was 13%. The rate of grade 3-4 adverse events (AEs) was 35%, and the overall response rate (ORR) was 19%. These results led to the premature termination of enrollment, as defined in the protocol stopping rules. The efficacy of the KRD combination in advanced-stage MCL patients who are R/R to BTKi is unsatisfactory and too toxic.

19.
Zhongguo Shi Yan Xue Ye Xue Za Zhi ; 32(3): 702-707, 2024 Jun.
Artigo em Chinês | MEDLINE | ID: mdl-38926956

RESUMO

OBJECTIVE: To investigate the effect of progression of disease within 24 months (POD24) on overall survival (OS) in patients with mantle cell lymphoma (MCL), and compare the clinical characteristics between POD24 and non-POD24 patients. METHODS: A retrospective analysis was performed on 50 MCL patients with treatment indications and regular treatment who were admitted to the Affiliated Hospital of Xuzhou Medical University from January 2010 to August 2020. According to the occurrence of POD24, the patients were grouped for prognostic evaluation and clinical characteristics comparison. RESULTS: Univariate Cox regression analysis showed that POD24, PLT, albumin, MIPI score, ECOG PS score, LDH were the factors influencing OS in newly diagnosed MCL patients (all P < 0.05). The results of multivariate Cox regression analysis showed that POD24ï¼»HR=16.797(95%CI : 3.671-76.861),P < 0.001ï¼½, albumin<40 g/Lï¼»HR=3.238(95%CI :1.095-9.572),P =0.034ï¼½ and ECOG PS score≥2ï¼»HR=4.005(95%CI :1.033-15.521),P =0.045ï¼½ were independent risk factors influencing OS in MCL patients. The incidence of PLT<100×109/L (33.3% vs 5.9%, P =0.033) and ECOG PS score ≥2 (45.5% vs 5.9%, P =0.040) were significantly higher in POD24 patients than those in non-POD24 patients. CONCLUSION: POD24 is an independent poor prognostic factor affecting the OS of MCL patients, and the patients with PLT<100×109/L and ECOG PS score≥2 at diagnosis have a higher probability of POD24.


Assuntos
Progressão da Doença , Linfoma de Célula do Manto , Humanos , Prognóstico , Estudos Retrospectivos , Masculino , Feminino , Taxa de Sobrevida , Modelos de Riscos Proporcionais , Pessoa de Meia-Idade
20.
Oncol Lett ; 28(2): 363, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38903699

RESUMO

Primary mantle cell lymphoma (MCL) in the gastrointestinal tract is rare, accounting for 4-9% of all reported cases of gastrointestinal non-Hodgkin lymphoma. Furthermore, involvement of the entire gastrointestinal tract in MCL is rare. The present report describes an example of MCL characterized by numerous diffuse polypoid lesions along the whole digestive tract. In particular, there was a focus on the endoscopic presentation of the digestive tract. The patient initially received a treatment regimen of rituximab combined with cyclophosphamide, doxorubicin, vincristine and prednisone. After two cycles of treatment, the regimen was changed to rituximab combined with etoposide, oxaliplatin and ifosfamide, with the addition of ibrutinib capsules. Patients with MCL have a poor prognosis; however, complete response can be achieved after treatment.

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