Introduction: Epstein-Barr virus (EBV) induced- hemophagocytic lymphohistiocytosis (HLH) is usually mediated through B cells. Herein, we describe our experience with a rare form of T and NK cell-mediated EBV-HLH.
Methods: A retrospective medical record analysis of children admitted with the diagnosis of T/NK cell-mediated EBV-HLH between 2010-2018 to the Children`s Healthcare of Atlanta medical center (GA, USA).
Results: The study included 10 patients (5 males, mean presenting age of 6.4 years; range: 1 to 19 years). Presentation consisted of fever, splenomegaly and coagulopathy in 9, 8 and 7 patients, respectively. Serum EBV DNA was identified in all the patients. In 5 patients, EBV was demonstrated in T or NK cells. NK cell activity was decreased or absent in 5 patients. High levels of soluble interleukin-2 receptor (≥2400 U/ml) were measured in 8 patients. CD3+CD8+CD5- T cell clone expansion was noted during workup. Treatment consisted of dexamethasone (n=10), etoposide (n=6) and cyclosporine A (n=3). Biological agents were given to 7 patients (rituximab (n=7), alemtuzumab (n=3) and anakinra (n=1). Intravenous immunoglobulins were given to 2 patients. Two patients underwent hematopoietic stem cell transplantation. Four out of 10 patients have died.
Conclusion: Early diagnosis and treatment of T/NK cell-mediated EBV-HLH is critical, as prognosis is often poor. CD5-CD8+ T cell expansion can be seen during immune workup of these patients.