Epstein-Barr Virus–Associated Nodal Marginal Zone Lymphoma: Part of the Spectrum of Posttransplant Lymphoproliferative Disorder?

2018 ◽  
Vol 27 (1) ◽  
pp. 94-97
Author(s):  
Chi Yuen Cheung ◽  
Wing Hung Lau ◽  
Wah Cheuk
Keyword(s):  
Lymphoproliferative Disorder ◽  
Epstein Barr Virus ◽  
Marginal Zone ◽  
Marginal Zone Lymphoma ◽  
Lymphoid Cells ◽  
Posttransplant Lymphoproliferative Disorder ◽  
Barr Virus ◽  
The Status ◽  
Epstein Barr ◽  
Nodal Marginal Zone Lymphoma

A 56-year-old man, who received deceased kidney transplant 20 years ago, presented with an enlarged submandibular lymph node. Histologic examination revealed nodal marginal zone lymphoma in which the neoplastic lymphoid cells showed diffuse positivity for Epstein-Barr virus early RNA by in situ hybridization. Systemic lymphoma workup showed stage I disease. The tumor was managed as a posttransplant lymphoproliferative disorder and the immunosuppression was modified. There was no evidence of lymphoma at follow-up 6 years after excision alone. This case supports the inclusion of Epstein-Barr virus–positive nodal marginal zone lymphoma as a form of monomorphic B-cell lymphoproliferative disorder, in line with the status of its extranodal mucosa-associated lymphoid tissue lymphoma counterpart.

scholarly journals Case Report: EBV-Positive Extra-Nodal Marginal Zone Lymphoma Associated With XMEN Disease Caused by a Novel Hemizygous Mutation in MAGT1

2021 ◽  
Vol 11 ◽  
Author(s):  
Xin Huang ◽  
Dan Liu ◽  
Zifen Gao ◽  
Cuiling Liu
Keyword(s):  
Epstein Barr Virus ◽  
Marginal Zone ◽  
Pediatric Population ◽  
Genetic Disorder ◽  
Marginal Zone Lymphoma ◽  
Epstein Barr Virus Infection ◽  
Loss Of Function ◽  
Barr Virus ◽  
Epstein Barr ◽  
Nodal Marginal Zone Lymphoma

BackgroundX-linked immunodeficiency with magnesium defect and Epstein-Barr virus infection and neoplasia (XMEN) disease is an X-linked genetic disorder of immune system caused by loss-of-function mutation in gene encoding Magnesium transporter 1 (MAGT1). Individuals with XMEN disease are prone to developing Epstein Barr Virus (EBV)-associated lymphomas. Herein, we report the first known case of an EBV+ EMZL associated with XMEN disease.Case presentationThe patient was an 8-year-old Chinese boy who suffered from recurrent infections from birth. Six months before, the patient presented with a painless mass on his upper lip and excisional biopsy revealed an EBV-positive extra-nodal marginal zone lymphoma (EBV+ EMZL). Furthermore, molecular investigations with next-generation sequencing identified a novel germline mutation in MAGT1 (c.828_829insAT) in the patient. The c.828_829insAT variant was predicted to cause premature truncation of MAGT1 (p.A277M.fs*11) and consequently was defined as likely pathogenic. The mutation was inherited from his asymptomatic heterozygous carrier mother. Hence the patient was diagnosed with an XMEN disease both clinically and genetically.ConclusionOur results expand the genetic spectrum of XMEN disease and also the clinical spectrum of EBV+ EMZL. We highlight the importance of the genetic etiology underlying EBV+ lymphoma in the pediatric population.

scholarly journals The potential usefulness of sputum cytology in the conclusive diagnosis of methotrexate-associated lymphoproliferative disorders: A case report

2019 ◽  
Vol 7 ◽  
pp. 2050313X1983601 ◽  
Author(s):  
Seiya Mizuguchi ◽  
Kenichi Mizutani ◽  
Manabu Yamashita ◽  
Hiroshi Minato ◽  
Sohsuke Yamada
Keyword(s):  
B Cell ◽  
Lymphoproliferative Disorder ◽  
Epstein Barr Virus ◽  
B Cell Lymphoma ◽  
Lymphoid Cells ◽  
Sputum Cytology ◽  
Barr Virus ◽  
Large B Cell Lymphoma ◽  
Epstein Barr ◽  
Conclusive Diagnosis

Background: Methotrexate has been used as an anchor drug for the treatment of rheumatoid arthritis and is considered to be a cause of methotrexate-associated lymphoproliferative disorder. Spontaneous regression can occur after withdrawal of methotrexate and may be associated with Epstein–Barr virus positivity and non-diffuse large B cell lymphoma histological type. Methotrexate-associated lymphoproliferative disorders are often diagnosed pathologically by lung biopsy. To the best of our knowledge, there have been no studies on the cytological diagnosis of methotrexate-associated lymphoproliferative disorder using sputum smears. Case: A 70-year-old man, who was diagnosed with rheumatoid arthritis 13 years previously and who had been treated with methotrexate, presented shortness of breath and productive cough. Methotrexate-associated lymphoproliferative disorder was suspected as the sputum cytology showed many atypical lymphoid cells with hyperchromatic enlarged nuclei, foamy cytoplasm and distinct nucleoli. Chest computed tomography revealed multiple nodular shadows with interstitial pneumonia in the bilateral lower lung field. A lung biopsy specimen contained atypical lymphoid cells that were immunohistochemically positive for CD20 and MUM-1, and weakly positive for bcl-6, but negative for CD3 and CD10. There were no Epstein–Barr virus-infectious lymphoid cells by ISH-EBER. He was finally diagnosed with methotrexate-associated lymphoproliferative disorder (non-germinal center B-cell-like diffuse large B cell lymphoma histological type). Most of the nodules disappeared spontaneously following the withdrawal of methotrexate. Discussion and conclusion: A cytologically conclusive diagnosis of methotrexate-associated lymphoproliferative disorder may be reached using sputum smears and clinical information.

Incidence, Risk Factors, Clinical Management, and Outcomes of Posttransplant Lymphoproliferative Disorder in Kidney Transplant Recipients

2019 ◽  
Vol 29 (2) ◽  
pp. 185-193 ◽  
Author(s):  
Michelle Liu ◽  
Shahid Husain ◽  
Olusegun Famure ◽  
Yanhong Li ◽  
S. Joseph Kim
Keyword(s):  
Risk Factors ◽  
Lymphoproliferative Disorder ◽  
Epstein Barr Virus ◽  
Transplant Recipients ◽  
Posttransplant Lymphoproliferative Disorder ◽  
Kidney Transplant Recipients ◽  
Barr Virus ◽  
Incidence Risk ◽  
Epstein Barr

Background: Posttransplant lymphoproliferative disorder (PTLD) is a severe complication after kidney transplantation. This study examined the incidence, risk factors, clinical management, and outcomes of PTLD in a cohort of kidney transplant recipients. Design: This single-center cohort study included 1642 patients transplanted from January 1, 2000, to December 31, 2012, with follow-up until December 31, 2013. The incidence and risk factors for PTLD were examined using a Cox proportional hazards model. A Cox model was also used to assess the association of PTLD and graft outcomes. Results: Sixteen recipients developed PTLD over follow-up. The incidence rate was 0.18 (95% confidence interval [CI]: 0.11-0.29) cases per 100 person-years. Four were from Epstein–Barr virus (EBV) mismatched (D+/R−) transplants and 12 from EBV-positive recipients (R+). Recipients with D+/R− matches were at a significantly higher risk of developing PTLD than R+ (hazard ratio [HR]: 7.52 [95% CI: 2.42-23.32]). Fifteen cases had immunosuppression reduced, 11 cases were supplemented with rituximab or ganciclovir, 6 cases required chemotherapy or radiation, and 6 cases had tumors excised. By the end of follow-up, 6 patients went into remission, 5 returned to chronic dialysis, and 5 patients died. Patients with PTLD were significantly more likely to have total graft failure (return to chronic dialysis, preemptive retransplant, or death with graft function) than patients without PTLD (HR: 3.41 [95% CI: 1.72-6.78). Discussion: Epstein–Barr virus mismatch continues to be a strong risk factor for developing PTLD after kidney transplantation. Recipients with PTLD have a poor prognosis, as the optimal management remains to be elucidated.

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