A case of intestinal perforation due to age-related EBV-associated B-cell lymphoproliferative disorder

Paisarn Boonsakan¹, Artit Jinawath¹, Kaettipong Kamprerasart¹, Weerapat Suwanthanma², Pantep Angchaisuksiri^3
1Department of Pathology, ²Department of Surgery, ³Division of Hematology, Department of Internal Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand

Background: Age-related Epstein-Barr virus (EBV)-associated lymphoproliferative disorder (LPD) is a recently novel EBV-positive clonal B-cell proliferation, occurring in patients >50 years old without any known immunodeficiency or prior lymphoma. The disease is believed to be related to immunological deterioration derived from the aging process and has aggressive clinical course.

Design: Case report.

Results: A 90-year-old Thai woman came to our hospital with fever and hematochezia. She finally developed bowel perforation. The histomorphologic, immunohistochemical, and molecular studies of smal bowel resection specimen supported the diagnosis of age-related EBV-associated LPD. Patient expired two months later due to infectious complication.

Conclusion: We report an unusual presentation of age-related EBV-associated LPD, which was confirmed by immunohistochemistry and molecular technique, discuss the updated clinicopathological data of the disease, and differential diagnosis of this entity from EBV-positive classical Hodgkin lymphoma.