Spontaneous resolution of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis.

Pediatr Blood Cancer

Division of Pediatric Hematology and Oncology, Department of Pediatrics, Duke University Medical Center, Durham, North Carolina 27710, USA.

Published: October 2010

Secondary hemophagocytic lymphohistiocytosis (sHLH) is a reactive, proliferative disorder of the immune system resulting in lymphohistiocytic proliferation, hemophagocytosis, and cytokine dysregulation. The most common infectious trigger in sHLH is Epstein-Barr virus (EBV-HLH). Current treatment protocols for EBV-HLH have a cure rate of approximately 75%; however, there are significant toxicities associated with these therapies. We present two patients with EBV-HLH who experienced spontaneous resolution of their disease prior to the initiation of therapy, suggesting there may be a subgroup of patients with EBV-HLH who do well with conservative management and can avoid potentially toxic therapies.

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http://dx.doi.org/10.1002/pbc.22618DOI Listing

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