Key result
Hepatic intravascular large B-cell lymphoma presents nonspecifically, leading to delayed diagnosis and dismal prognosis.
Why the study?
Epstein-Barr virus-positive intravascular large B-cell lymphoma in the liver had never been reported in the literature.
Case Report (n=13)
No
This case report describes the first known case of EBV-positive intravascular large B-cell lymphoma of the liver, highlighting its nonspecific manifestations and aggressive clinical course.
May prompt biopsy consideration in unexplained hepatopathy; first EBV-positive case extends spectrum but leaves management open.
BACKGROUND: Intravascular large B-cell lymphoma (IVLBCL) is an extremely rare subtype of diffuse large B-cell lymphoma that most commonly involves the central nervous system, skin, and bone marrow. To our knowledge, Epstein-Barr virus (EBV)-positive IVLBCL in the liver has never been reported in the literature. CASE PRESENTATION: We report a case of a 65-year-old Chinese man with complaint of fever for 18 days. No obvious abnormality was found by physical examination. Laboratory findings were notable for anemia, thrombocytopenia, and elevated level of serum lactate dehydrogenase. Bone marrow on smear, biopsy, and flow cytometry revealed no lymphoma. Imaging studies showed a slightly lower density lesion in the liver with high fluorodeoxyglucose uptake and hepatosplenomegaly. Percutaneous liver biopsy revealed clustering of large atypical lymphocytes within the hepatic sinusoids. Immunohistochemically, these lymphoma cells were positive for CD20, PAX-5, MUM-1, BCL-6 and CD5, but negative for CD3 and CD10. Besides, Epstein-Barr virus-encoded RNA was detected in tumor cells by in situ hybridization. BCL-2, BCL-6 and MYC genes were intact tested by fluorescence in situ hybridization analysis. The patient was diagnosed as IVLBCL and died after 1 month of hospitalization without receiving immunochemotherapy. CONCLUSIONS: IVLBCL of the liver is a highly rare lymphoma with nonspecific manifestations and dismal prognosis. Full recognition of its clinicopathological features will help to better diagnose this disease.
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Li et al. (2020) conducted a case report in Intravascular large B-cell lymphoma of the liver (n=13). Intravascular large B-cell lymphoma of the liver is an extremely rare and aggressive disease with nonspecific manifestations, often leading to delayed diagnosis and a dismal prognosis.
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