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.
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.
Authors: T Weber; T Ettrich; M Christopeit; A Lindner; H J Holzhausen; A Oehme; D Arnold; H H Wolf; C Lübbert; A S Kekulé; H J Schmoll; K Werdan; H Ebelt Journal: Internist (Berl) Date: 2012-01 Impact factor: 0.743
Authors: Theodoros Kelesidis; Romney Humphries; Dawn Terashita; Shahrooz Eshaghian; Mary C Territo; Jonathan Said; Michael Lewinski; Judith S Currier; David Pegues Journal: J Med Virol Date: 2012-05 Impact factor: 2.327
Authors: Jeffrey I Cohen; Julie E Niemela; Jennifer L Stoddard; Stefania Pittaluga; Helen Heslop; Elaine S Jaffe; Kennichi Dowdell Journal: J Clin Immunol Date: 2015-05-07 Impact factor: 8.317
Authors: Michael B Jordan; Carl E Allen; Sheila Weitzman; Alexandra H Filipovich; Kenneth L McClain Journal: Blood Date: 2011-08-09 Impact factor: 22.113
Authors: Jan Rohr; Karin Beutel; Andrea Maul-Pavicic; Thomas Vraetz; Jens Thiel; Klaus Warnatz; Ilka Bondzio; Ute Gross-Wieltsch; Michael Schündeln; Barbara Schütz; Wilhelm Woessmann; Andreas H Groll; Brigitte Strahm; Julia Pagel; Carsten Speckmann; Gritta Janka; Gillian Griffiths; Klaus Schwarz; Udo zur Stadt; Stephan Ehl Journal: Haematologica Date: 2010-09-07 Impact factor: 9.941
Authors: Udo zur Stadt; Jan Rohr; Wenke Seifert; Florian Koch; Samantha Grieve; Julia Pagel; Julia Strauss; Brigitte Kasper; Gudrun Nürnberg; Christian Becker; Andrea Maul-Pavicic; Karin Beutel; Gritta Janka; Gillian Griffiths; Stephan Ehl; Hans Christian Hennies Journal: Am J Hum Genet Date: 2009-10 Impact factor: 11.025