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Published on: March 30, 2018
A mild, self-resolving case of Epstein-Barr virus-induced hemophagocytic lymphohistiocytosis
Biplov Adhikari1, Shiavax J Rao1, Christopher J Haas2,3
1Department of Medicine, MedStar Health Internal Medicine Residency Program, MedStar Union Memorial Hospital, Baltimore, MD, the United States of America.
Hemophagocytic lymphohistiocytosis (HLH) is a multisystem disease caused by an excessive activation of the immune system. In most instances, HLH can be fatal without treatment; a life-threatening syndrome driven by a dysregulated immune system and activation of macrophages resulting in cytokine release and consequent cellular damage. HLH can occur as a consequence of multiple genetic abnormalities or environmental triggers. We present an interesting case of mild, self-resolving, HLH due to Epstein-Barr Virus (EBV) infection in a young woman. The best-known diagnostic criteria are based on the HLH-2004 trial, incorporating either the presence of known mutations or five of eight clinical and laboratory findings. Prompt initiation of etoposide-containing therapy is associated with improved survival. Rituximab, an anti-CD20 antibody, can also remove EBV-harboring B-cells and improve outcomes. In a rare subset of patients, the disease can spontaneously resolve without any therapeutic interventions thus sparing the patients from toxic therapies.
Hemophagocytic lymphohistiocytosis (HLH) is a multisystem disease caused by an excessive activation of the immune system. In most instances, HLH can be fatal without treatment; a life-threatening syndrome driven by a dysregulated immune system and activation of macrophages resulting in cytokine release and consequent cellular damage. HLH can occur as a consequence of multiple genetic abnormalities or environmental triggers. We present an interesting case of mild, self-resolving, HLH due to Epstein-Barr Virus (EBV) infection in a young woman. The best-known diagnostic criteria are based on the HLH-2004 trial, incorporating either the presence of known mutations or five of eight clinical and laboratory findings. Prompt initiation of etoposide-containing therapy is associated with improved survival. Rituximab, an anti-CD20 antibody, can also remove EBV-harboring B-cells and improve outcomes. In a rare subset of patients, the disease can spontaneously resolve without any therapeutic interventions thus sparing the patients from toxic therapies.
