A rare presentation of EBV+ mucocutaneous ulcer that led to a diagnosis of hypogammaglobulinemia

Kleinman, Sara H.; Jhaveri, Devi; Caimi, Paolo; Cameron, Robert P.; Lemonovich, Tracy L.; Meyerson, Howard; Hostoffer, Robert; Tcheurekdjian, Haig

doi:10.1016/j.jaip.2014.07.009

Cited by 24 publications

(36 citation statements)

References 9 publications

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“…There were some differences in the predisposing factors in the EBVMCU patients in Western versus Eastern countries. Specifically, EBVMCU was associated with iatrogenic immunosuppressive drugs in 38% of Western patients, with age‐related immunosenescence in 36%, and with transplantation and other factors in 26% . In Eastern countries, EBVMCU was reported in the setting of iatrogenic immunosuppressive drug use for autoimmune disease in 59% of patients, post‐chemotherapy in 36%, and in association with other factors in 5% .…”

Section: Discussionmentioning

confidence: 99%

“…Specifically, EBVMCU was associated with iatrogenic immunosuppressive drugs in 38% of Western patients, with age-related immunosenescence in 36%, and with transplantation and other factors in 26%. 2,3,5,6,10,11,[14][15][16][17][18][19][20][21][22][23][24][25][26] In Eastern countries, EBVMCU was reported in the setting of iatrogenic immunosuppressive drug use for autoimmune disease in 59% of patients, post-chemotherapy in 36%, and in association with other factors in 5%. 4,12,[27][28][29] The prevalence of autoimmune disease, e.g.…”

Section: Discussionmentioning

confidence: 99%

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Comparison of Epstein–Barr virus‐positive mucocutaneous ulcer associated with treated lymphoma or methotrexate in Japan

et al. 2018

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Comparison of Epstein–Barr virus‐positive mucocutaneous ulcer associated with treated lymphoma or methotrexate in Japan

et al. 2018

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“…In Because no diagnostic criteria have been established yet for EBV-MCU, it is diagnosed on the basis of clinical background factors and histopathological findings [2][3][4][5][6][7][8][9][10][11][12][13]. It has been reported that the characteristic histopathological findings of EBV-MCU include infiltration of various cells, various immunohistochemical patterns such as CD20 and CD30 positivity, the detection of EBER-positive cells by ISH, among which HRS-like B cells are found [2][3][4][5][6][7][8][9][10][11][12][13]. In the present case, the initial histopathological findings of the biopsy specimen collected from the lesion were nonspecific, which made the diagnosis difficult.…”

Section: Discussionmentioning

confidence: 99%

“…Epstein-Barr virus-positive mucocutaneous ulcer (EBV-MCU) is a new category of mature B-cell neoplasms and was formally adopted in the World Health Organization (WHO) classification of lymphoid neoplasms updated in 2016 [1]. The results of previous studies suggest that the onset of EBV-MCU is related to the decreased immunity of the patient, the causes of which include primary or acquired immunosuppression, iatrogenic immunosuppression by immunosuppressive agents, and aging [2][3][4][5][6][7][8][9][10][11][12][13]. Ulcers occur in the oropharyngeal mucosa, skin, and gastrointestinal tract.…”

Section: Introductionmentioning

confidence: 99%

A rare case of Epstein–Barr virus-positive mucocutaneous ulcer that developed into an intestinal obstruction: a case report

et al. 2020

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Background: Epstein-Barr virus-positive mucocutaneous ulcer (EBV-MCU) is a new category of mature B-cell neoplasms. Ulcers occur in the oropharyngeal mucosa, skin, and gastrointestinal tract. The onset of EBV-MCU is suggested to be related to the decreased immunity of the patient, the causes of which include the use of immunosuppressive agents and aging. EBV-MCU may regress spontaneously and it often has a benign course after the dose reduction or discontinuation of immunosuppressive agents or during follow-up. Here, we report the case of a patient who required surgical resection for the intestinal obstruction arising from EBV-MCU. Case presentation: A Japanese elderly male visited our hospital with chief complaints of a palpable mass and dull pain in the left upper quadrant, loss of appetite, and weight loss. Although abdominal computed tomography and total colonoscopy (TCS) revealed a tumor with circumferential ulcer in the transverse colon, histopathological analysis of a biopsy specimen of this lesion showed only nonspecific inflammation. Because the tumor spontaneously regressed during the time he underwent tests to obtain a second opinion from another hospital, TCS was reperformed on the patient. TCS revealed that the tumor decreased in size and the inflammatory changes in the surrounding mucosa tended to improve; however, tightening of the surrounding mucosa due to scarring was observed. Another histopathological analysis of a biopsy specimen showed widespread erosion of the mucosa and the formation of granulation tissue with marked infiltration of various inflammatory cells into the mucosal tissue of the large intestine. Moreover, some of the B-lymphocyte antigen CD20positive B cells were also positive for EBV-encoded small RNA-1, suggesting the possibility of EBV-MCU. Later, the tumor developed into an intestinal obstruction; thus, the transverse colon was resected. Histopathological analysis of the resected specimen demonstrated scattered Hodgkin and Reed-Sternberg-like multinucleated large B cells in addition to EBER-1positive cells. The patient was finally diagnosed as having EBV-MCU. Conclusions: This is the first report of a case of EBV-MCU that developed into an intestinal obstruction requiring surgical resection. It is necessary to consider the possibility of EBV-MCU when examining an ulcerative or tumorous lesion in the gastrointestinal tract.

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“…Lymphoma is more common in CVID than the general population, leading to suggestions for a significant role of viruses with oncogenic potential . There are cases of malignancy in antibody‐deficient patients with evidence of EBV in tissue samples . However, there is limited evidence that EBV‐related malignancy is more common in CVID patients, with instead a preponderance of extranodal EBV‐negative B cell lymphomas .…”

Section: Herpesvirusesmentioning

confidence: 99%

Viral infection in primary antibody deficiency syndromes

Jones

Buckland

Breuer

et al. 2019

Reviews in Medical Virology

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Patients with primary antibody deficiency syndromes such as X-linked agammaglobulinemia (XLA) and common variable immunodeficiency (CVID) are at increased risk of severe and invasive infection. Viral infection in these populations has been of increasing interest as evidence mounts that viruses contribute significant morbidity and mortality: this is mediated both directly and via aberrant immune responses.We explain the importance of the humoral immune system in defence against viral pathogens before highlighting several significant viral syndromes in patients with antibody deficiency.We explore historical cases of hepatitis C via contaminated immunoglobulin products, the predisposition to invasive enteroviral infections, prolonged excretion of vaccinederived poliovirus, the morbidity of chronic norovirus infection, and recent literature revealing the importance of respiratory viral infections. We discuss evidence that herpesviruses may play a role in driving the inflammatory disease seen in a subset of patients. We explore the phenomenon of within-host evolution during chronic viral infection and the potential emergence of new pathogenic strains. We highlight novel and emerging viruses identified via deep sequencing techniques. We describe the treatment strategies that have been attempted in all these scenarios and the urgent outstanding questions for research.

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A rare presentation of EBV+ mucocutaneous ulcer that led to a diagnosis of hypogammaglobulinemia

Cited by 24 publications

References 9 publications

Comparison of Epstein–Barr virus‐positive mucocutaneous ulcer associated with treated lymphoma or methotrexate in Japan

Comparison of Epstein–Barr virus‐positive mucocutaneous ulcer associated with treated lymphoma or methotrexate in Japan

A rare case of Epstein–Barr virus-positive mucocutaneous ulcer that developed into an intestinal obstruction: a case report

Viral infection in primary antibody deficiency syndromes

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