Infection of T Lymphocytes in Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis in Children of Non-Asian Origin

Background. Epstein-Barr virus (EBV) is one of the most frequent triggers of hemophagocytic lymphohistiocytosis EBV-associated HLH (EBV-HLH) and ectopic infection of T cells has been particularly described in patients from Far East Asia. Procedure. In a cohort of 12 children with EBV-HLH treated in Germany, the EB viral load was detected by real-time polymerase chain reaction in plasma and peripheral blood mononuclear cells (PBMC). Virological and clinical data were analyzed retrospectively. Results. Among the 12 mainly German patients, children with underlying immunodeficiencies as well as otherwise healthy individuals were affected. The clinical Course ranged from a steroid-responding to a fatal disease despite intensive treatment. Increased EBV copy numbers in plasma and/or PBMC were found in all patients. Serial measurements reflected the course of the disease. Cell-type specific viral load was determined in seven patients and revealed EBV-infection of T cells in all of them. in contrast to the reported Asian patients a significant viral load was also found in B cells. Conclusions. T cell infection appears to be a typical feature of EBV-associated HLH irrespective of patients ethnic background and the clinical Course. Evaluation of cell-type specific infection should be considered when targeted therapy is applied. Pediatr Blood Cancer 2009;53:184-190. (C) 2009 Wiley-Liss, Inc.