Literature DB >> 21953027

[Unexplained fever and B-symptoms in a young male Black African].

T Weber1, 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.   

Abstract

An immunocompetent Nigerian developed a fulminant hemophagocytic lymphohistiocytosis due to Epstein-Barr virus reactivation. The patient initially presented with fever, hepatosplenomegaly and pancytopenia. The clinical status of our patient deteriorated quickly despite treatment with corticoids. Escalation of immunosuppressive treatment was not possible. He died of lung, liver and circulatory failure in our intensive care unit.Hemophagocytic lymphohistiocytosis is a rare disease characterized by inflammation due to prolonged and excessive activation of antigen-presenting cells. High plasma ferritin levels and phagocytosis of hematopoetic cells in bone marrow, spleen and liver lead to the diagnosis. Hemophagocytic lymphohistiocytosis should therefore be included in the differential diagnosis in patients with persistent fever, hepatosplenomegaly and cytopenia.

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Year:  2012        PMID: 21953027     DOI: 10.1007/s00108-011-2912-6

Source DB:  PubMed          Journal:  Internist (Berl)        ISSN: 0020-9554            Impact factor:   0.743


  12 in total

1.  Treatment of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis (EBV-HLH); update 2010.

Authors:  Shinsaku Imashuku
Journal:  J Pediatr Hematol Oncol       Date:  2011-01       Impact factor: 1.289

2.  HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis.

Authors:  Jan-Inge Henter; Annacarin Horne; Maurizio Aricó; R Maarten Egeler; Alexandra H Filipovich; Shinsaku Imashuku; Stephan Ladisch; Ken McClain; David Webb; Jacek Winiarski; Gritta Janka
Journal:  Pediatr Blood Cancer       Date:  2007-02       Impact factor: 3.167

3.  Hemophagocytic lymphohistiocytosis (HLH) and related disorders.

Authors:  Alexandra H Filipovich
Journal:  Hematology Am Soc Hematol Educ Program       Date:  2009

4.  Infection of T lymphocytes in Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in children of non-Asian origin.

Authors:  Karin Beutel; Ute Gross-Wieltsch; Thomas Wiesel; Udo Zur Stadt; Gritta Janka; Hans-Joachim Wagner
Journal:  Pediatr Blood Cancer       Date:  2009-08       Impact factor: 3.167

Review 5.  X-linked lymphoproliferative disease: pathology and diagnosis.

Authors:  D M Maia; C P Garwacki
Journal:  Pediatr Dev Pathol       Date:  1999 Jan-Feb

6.  Hemophagocytic lymphohistiocytosis presenting with nonimmune hydrops fetalis.

Authors:  Colleen Ann Malloy; Carol Polinski; Serhan Alkan; Ricarchito Manera; Malliswari Challapalli
Journal:  J Perinatol       Date:  2004-07       Impact factor: 2.521

7.  Poor outcomes of chronic active Epstein-Barr virus infection and hemophagocytic lymphohistiocytosis in non-Japanese adult patients.

Authors:  Gabe S Sonke; Inge Ludwig; Hannah van Oosten; Joke W Baars; Ellen Meijer; Arnon P Kater; Daphne de Jong
Journal:  Clin Infect Dis       Date:  2008-07-01       Impact factor: 9.079

8.  Severe Epstein-Barr virus-associated hemophagocytic syndrome in six adult patients.

Authors:  Anat Scheiman Elazary; Dana G Wolf; Gail Amir; Batia Avni; Deborah Rund; Dina Ben Yehuda; Sigal Sviri
Journal:  J Clin Virol       Date:  2007-08-20       Impact factor: 3.168

9.  Multiple organ failure and severe bone marrow dysfunction in two 18 year-old Caucasian patients: Epstein-Barr virus and the haemophagocytic syndrome.

Authors:  P A Berry; W Bernal; A Pagliuca; E Sizer; J R Salisbury; J A Wendon; G Auzinger
Journal:  Anaesthesia       Date:  2008-08-19       Impact factor: 6.955

10.  Nationwide survey of hemophagocytic lymphohistiocytosis in Japan.

Authors:  Eiichi Ishii; Shouichi Ohga; Shinsaku Imashuku; Masaki Yasukawa; Hiroyuki Tsuda; Ikuo Miura; Ken Yamamoto; Hisanori Horiuchi; Kenzo Takada; Koichi Ohshima; Shigeo Nakamura; Naoko Kinukawa; Kazuo Oshimi; Keisei Kawa
Journal:  Int J Hematol       Date:  2007-07       Impact factor: 2.490

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