EBV-positive intravascular large B-cell lymphoma of the small intestine: a case report and literature review

Abstract

Abstract Background: Intravascular large B-cell lymphoma is a rare lymphoma that causes symptoms in the central nervous system, skin, and bone marrow. Case presentation: We report a 75-year-old man who was admitted to the hospital with abdominal pain for four hours. Physical examination revealed abdominal pain and mottled skin. Laboratory tests revealed thrombocytopenia and elevated levels of lactate dehydrogenase.Abdominal CT examination showed thickening, edema, and necrosis of the small intestine wall. Surgical excision of the necrotic small bowel revealed numerous small rounds, consistent, atypical lymphocytes in the mesenteric vein. These cells were positive for CD20, CD79a, CD10, and BCL-2, and were positive for Epstein Barr virus-encoded small RNA (EBER) by in situ hybridization. The patient was diagnosed with IVLBCL and died of MODS after a week of hospitalization without chemotherapy. Conclusions: IVLBCL that occurs in the small intestine and even in the gastrointestinal tract is an exceedingly rare disease with insidious onset, rapid disease progression and poor prognosis. Fully understanding its clinicopathological features is helpful to understand the disease, make an early diagnosis, and prevent the rapid deterioration of the disease.