Spontaneous resolution of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis.
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.
Belyea
Brian
B
Division of Pediatric Hematology and Oncology, Department of Pediatrics, Duke University Medical Center, Durham, North Carolina 27710, USA. brian.belyea@duke.edu
Hinson
Ashley
A
Moran
Cassandra
C
Hwang
Eugene
E
Heath
Jessica
J
Barfield
Raymond
R
D000293:Adolescent; D000328:Adult; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D005260:Female; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis*; D008297:Male