Epstein Virus Barr-Positive Diffuse Large B-Cell Lymphoma Associated with Hemophagocytic Lymphohistiocytosis. Issue 8 (10th July 2020)

• Record Type: Journal Article
• Authors: Ricard, Jocelyn A; Charles, River; Tommee, Carolina Gil; Yohe, Sophia; Bell, W Robert; Flanagan, Margaret E
• Is Part Of: Journal of neuropathology and experimental neurology. Volume 79:Issue 8(2020)
• Page Start: 915
• Page End: 920
• Publication Date: 2020-07-10
• Subjects: Epstein Barr virus (EBV) -- Hemophagocytic lymphohistiocytosis (HLH); Neurology -- Diseases -- Periodicals; Neurology -- Diseases -- Physiopathology -- Periodicals; 616.8047
• DOI: 10.1093/jnen/nlaa061
• Languages: English
• ISSNs: 0022-3069

Abstract

Abstract: Hemophagocytic lymphohistiocytosis (HLH) is a rare and often fatal disease if not diagnosed and treated promptly. HLH can be due to genetic factors or infections, malignancies and collagen-associated vascular diseases. Malignancy-associated HLH is not only more common in the setting of T/NK-cell lymphomas, but may also rarely be seen in the setting of B-cell lymphoma. Here, we describe a unique case of a patient who initially was diagnosed with HLH secondary to Epstein Barr virus (EBV) infection and subsequently developed EBV-positive diffuse large B-cell lymphoma affecting the brain. This case highlights the spectrum of findings associated with EBV infections and the challenges in diagnosing underlying diseases associated with HLH.