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.

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 Epstein-Barr Virus Infection and Thiopurine Therapy in a Pediatric Population with Inflammatory Bowel Disease

2020 ◽  
Vol 27 (5) ◽  
pp. 318-323
Author(s):  
Maria Do Céu Espinheira ◽  
Isabel Pinto Pais ◽  
Ivete Afonso ◽  
Jorge Ferreira ◽  
Eunice Trindade ◽  
...  
Keyword(s):  
Inflammatory Bowel Disease ◽  
Virus Infection ◽  
Epstein Barr Virus ◽  
Pediatric Population ◽  
Epstein Barr Virus Infection ◽  
Barr Virus ◽  
Thiopurine Therapy ◽  
Inflammatory Bowel ◽  
Epstein Barr ◽  
Barr Virus Infection

scholarly journals Acute Acalculous Cholecystitis from Infection with Epstein–Barr Virus in a Previously Healthy Child: A Case Report

2020 ◽  
Vol 4 (3) ◽  
pp. 393-396
Author(s):  
Robert Langenohl ◽  
Scott Young ◽  
Kyle Couperus
Keyword(s):  
Case Report ◽  
Epstein Barr Virus ◽  
Pediatric Population ◽  
Bacterial Overgrowth ◽  
Acalculous Cholecystitis ◽  
Operative Management ◽  
Acute Acalculous Cholecystitis ◽  
Epstein Barr Virus Infection ◽  
Barr Virus ◽  
Epstein Barr

Background: Acute cholecystitis is the acute inflammation of the gallbladder. In adults it is most frequently caused by a gallstone(s) obstructing outflow from the cystic duct, leading to gallbladder distention and edema with eventual development of biliary stasis and bacterial overgrowth, often requiring operative management. However, in children acalculous cholecystitis is more common and is often the result of an infectious process. Case Report: Here we present a case of acute acalculous cholecystitis caused by infection with Epstein-Barr virus in an otherwise healthy three-year-old male. Conclusion: Acalculous cholecystitis is an uncommon but potentially significant complication of Epstein-Barr virus infection in the pediatric population. Emergency providers should consider this diagnosis in any child being evaluated for EBV with the complaint of abdominal pain.

scholarly journals Acute acalculous cholecystitis by Epstein-Barr virus infection: a rare association

2015 ◽  
Vol 7 (4) ◽  
Author(s):  
Liliana Branco ◽  
Maria Vieira ◽  
Cristiana Couto ◽  
Maria D. Coelho ◽  
Carla Laranjeira
Keyword(s):  
Epstein Barr Virus ◽  
Pediatric Population ◽  
Rare Complication ◽  
Acalculous Cholecystitis ◽  
Acute Acalculous Cholecystitis ◽  
Epstein Barr Virus Infection ◽  
Barr Virus ◽  
Ebv Infection ◽  
Epstein Barr ◽  
Barr Virus Infection

Acute acalculous cholecystitis (AAC) is a rare complication of Epstein Barr virus (EBV) infection, with only a few cases reported among pediatric population. This clinical condition is frequently associated with a favorable outcome and, usually, a surgical intervention is not required. We report a 16-year-old girl who presented with AAC following primary EBV infection. The diagnosis of AAC was documented by clinical and ultrasonographic examination, whereas EBV infection was confirmed serologically. A conservative treatment was performed, with a careful monitoring and serial ultrasonographic examinations, which led to the clinical improvement of the patient. Pediatricians should be aware of the possible association between EBV and AAC, in order to offer the patients an appropriate management strategy.

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