Infection of T lymphocytes in Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in children of non-Asian origin - PubMed (original) (raw)
Infection of T lymphocytes in Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in children of non-Asian origin
Karin Beutel et al. Pediatr Blood Cancer. 2009 Aug.
Abstract
Background: Epstein-Barr virus (EBV) is one of the most frequent triggers of hemophagocytic lymphohistiocytosis (HLH). 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.
(c) 2009 Wiley-Liss, Inc.
Comment in
- Infection of T lymphocytes in non-Asian patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis.
Imashuku S. Imashuku S. Pediatr Blood Cancer. 2009 Dec 15;53(7):1359; author reply 1360. doi: 10.1002/pbc.22118. Pediatr Blood Cancer. 2009. PMID: 19533652 No abstract available.
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