Epstein Virus Barr-Positive Diffuse Large B-Cell Lymphoma Associated with Hemophagocytic Lymphohistiocytosis. 2020

Jocelyn A Ricard, and River Charles, and Carolina Gil Tommee, and Sophia Yohe, and W Robert Bell, and Margaret E Flanagan
From the Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis, Minnesota.

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.

UI MeSH Term Description Entries
D008297 Male Males
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D016403 Lymphoma, Large B-Cell, Diffuse Malignant lymphoma composed of large B lymphoid cells whose nuclear size can exceed normal macrophage nuclei, or more than twice the size of a normal lymphocyte. The pattern is predominantly diffuse. Most of these lymphomas represent the malignant counterpart of B-lymphocytes at midstage in the process of differentiation. Diffuse Large B-Cell Lymphoma,Diffuse, Large B-Cell, Lymphoma,Histiocytic Lymphoma, Diffuse,Lymphoma, Histiocytic, Diffuse,Diffuse Large-Cell Lymphoma,Histiocytic Lymphoma,Large Lymphoid Lymphoma, Diffuse,Large-Cell Lymphoma, Diffuse,Lymphoma, Diffuse Large-Cell,Lymphoma, Histiocytic,Lymphoma, Large Cell, Diffuse,Lymphoma, Large Lymphoid, Diffuse,Lymphoma, Large-Cell, Diffuse,Diffuse Histiocytic Lymphoma,Diffuse Histiocytic Lymphomas,Diffuse Large B Cell Lymphoma,Diffuse Large Cell Lymphoma,Diffuse Large-Cell Lymphomas,Histiocytic Lymphomas,Large Cell Lymphoma, Diffuse,Lymphoma, Diffuse Histiocytic,Lymphoma, Diffuse Large Cell
D051359 Lymphohistiocytosis, Hemophagocytic A group of related disorders characterized by LYMPHOCYTOSIS; HISTIOCYTOSIS; and hemophagocytosis. The two major forms are familial and reactive. Familial Hemophagocytic Lymphocytosis,Hemophagocytic Lymphohistiocytosis, Familial,Hemophagocytic Syndrome, Infection-Associated,Hemophagocytic Syndrome, Reactive,Hemophagocytic Syndromes,Reactive Hemophagocytic Syndrome,Erythrophagocytic Lymphohistiocytosis, Familial,Familial Erythrophagocytic Lymphohistiocytosis,Familial Hemophagocytic Histiocytosis,Familial Hemophagocytic Lymphohistiocytosis,Familial Hemophagocytic Reticulosis,Familial Histiocytic Reticulosis,Hemophagocytic Lymphohistiocytosis Familial -1,Hemophagocytic Lymphohistiocytosis, Familial, 1,Hemophagocytic Reticulosis, Familial,Hemophagocytic Syndrome,Primary Hemophagocytic Hymphohistiocytosis,Primary Hemophagocytic Lymphohistiocytosis,Reticulosis, Familial Histiocytic,Erythrophagocytic Lymphohistiocytoses, Familial,Familial Erythrophagocytic Lymphohistiocytoses,Familial Hemophagocytic Histiocytoses,Familial Hemophagocytic Lymphocytoses,Familial Hemophagocytic Lymphohistiocytoses,Familial Hemophagocytic Reticuloses,Familial Histiocytic Reticuloses,Hemophagocytic Histiocytoses, Familial,Hemophagocytic Histiocytosis, Familial,Hemophagocytic Hymphohistiocytoses, Primary,Hemophagocytic Hymphohistiocytosis, Primary,Hemophagocytic Lymphocytoses, Familial,Hemophagocytic Lymphocytosis, Familial,Hemophagocytic Lymphohistiocytoses,Hemophagocytic Lymphohistiocytoses, Familial,Hemophagocytic Lymphohistiocytoses, Primary,Hemophagocytic Lymphohistiocytosis,Hemophagocytic Lymphohistiocytosis Familial 1,Hemophagocytic Lymphohistiocytosis, Primary,Hemophagocytic Reticuloses, Familial,Hemophagocytic Syndrome, Infection Associated,Histiocytic Reticuloses, Familial,Histiocytic Reticulosis, Familial,Histiocytoses, Familial Hemophagocytic,Histiocytosis, Familial Hemophagocytic,Hymphohistiocytoses, Primary Hemophagocytic,Hymphohistiocytosis, Primary Hemophagocytic,Infection-Associated Hemophagocytic Syndrome,Lymphocytoses, Familial Hemophagocytic,Lymphocytosis, Familial Hemophagocytic,Lymphohistiocytoses, Familial Erythrophagocytic,Lymphohistiocytoses, Familial Hemophagocytic,Lymphohistiocytoses, Hemophagocytic,Lymphohistiocytoses, Primary Hemophagocytic,Lymphohistiocytosis, Familial Erythrophagocytic,Lymphohistiocytosis, Familial Hemophagocytic,Lymphohistiocytosis, Primary Hemophagocytic,Primary Hemophagocytic Hymphohistiocytoses,Primary Hemophagocytic Lymphohistiocytoses,Reticuloses, Familial Hemophagocytic,Reticuloses, Familial Histiocytic,Reticulosis, Familial Hemophagocytic
D020031 Epstein-Barr Virus Infections Infection with human herpesvirus 4 (HERPESVIRUS 4, HUMAN); which may facilitate the development of various lymphoproliferative disorders. These include BURKITT LYMPHOMA (African type), INFECTIOUS MONONUCLEOSIS, and oral hairy leukoplakia (LEUKOPLAKIA, HAIRY). EBV Infections,Epstein-Barr Virus Infection,Herpesvirus 4 Infections, Human,Human Herpes Virus 4 Infections,Human Herpesvirus 4 Infections,Infections, EBV,Infections, Epstein-Barr Virus,EBV Infection,Epstein Barr Virus Infection,Epstein Barr Virus Infections,Infection, EBV,Infection, Epstein-Barr Virus,Virus Infection, Epstein-Barr,Virus Infections, Epstein-Barr

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