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Case Reports in Rheumatology
Volume 2018, Article ID 5364985, 4 pages
https://doi.org/10.1155/2018/5364985
Case Report

Intravascular Large B-Cell Lymphoma Mimicking Temporal Arteritis

Department of Pathology, Medstar Georgetown University Hospital, Washington, DC, USA

Correspondence should be addressed to Ifeyinwa Emmanuela Obiorah; ude.nwotegroeg.tenug@haroibo.e.awniyefi

Received 29 November 2017; Accepted 1 April 2018; Published 29 April 2018

Academic Editor: Shigeko Inokuma

Copyright © 2018 Ifeyinwa Emmanuela Obiorah and Metin Ozdemirli. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Intravascular lymphoma is a rare type of lymphoma, characterized by growth of lymphoma cells within the microvasculature. The majority of the cases are of B-cell lineage, although rare examples of T or NK lineage have also been reported. The lymphoma is usually widely disseminated in the vascular spaces of any organ at the time of diagnosis including the skin and bone marrow. Lymph nodes are typically spared. The clinical picture depends on the specific organ involvement making the correct diagnosis very difficult. Here, we report a case of intravascular large B-cell lymphoma diagnosed postmortem on a 69-year-old African-American male who presented with unilateral proptosis and visual loss. An initial diagnosis of temporal arteritis was made and the patient received corticosteroids. However, the patient developed multiorgan failure and expired. On autopsy, there was disseminated intravascular lymphoma involving predominantly vessels within the heart, kidneys, liver, stomach, lungs, adrenal glands, small intestine, bladder, thyroid, and brain. Interestingly, there was also partial involvement of the retroperitoneal lymph nodes which is an unusual presentation in this disorder. Immunohistochemical staining showed that the lymphoma cells were positive for CD20, indicating B-cell phenotype. This case supports the “mimicking nature” of this rare entity with an unusual presentation with proptosis and visual loss, simulating temporal arteritis and a rare involvement of the retroperitoneal lymph nodes. The presentation of intravascular large B-cell lymphoma can vary, and the key to diagnosis is dependent on histopathology and immunohistochemistry. Increased awareness, early tissue diagnosis, and prompt chemotherapy are crucial for this otherwise lethal disease.