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Epstein‐Barr virus induced virus‐associated hemophagocytic syndrome and monoclonal TCR‐beta rearrangement: a case report
Author(s) -
Bird George,
Peel Darryl,
McCarthy Keith,
Williams Hugh
Publication year - 1997
Publication title -
hematological oncology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.918
H-Index - 44
eISSN - 1099-1069
pISSN - 0278-0232
DOI - 10.1002/(sici)1099-1069(199702)15:1<47::aid-hon597>3.0.co;2-1
Subject(s) - virus , medicine , gene rearrangement , monoclonal , t cell receptor , virology , pancytopenia , epstein–barr virus , histiocyte , bone marrow , immunology , monoclonal antibody , biology , t cell , gene , antibody , immune system , biochemistry
Virus‐associated hemophagocytic syndrome (VAHS) is a rare histiocytic proliferative disorder secondary to viral infection affecting children and young adults and in which there is both a poor prognosis and no specific treatment. We report a case of VAHS in a 22‐year‐old woman secondary to infection with Epstein Barr virus in which multiorgan failure developed with a fatal outcome 3 weeks after presentation. Clonal analysis showed EBV incorporation into the T cell genome accompanied by monoclonal rearrangement of the TCR beta gene. Treatment with cyclosporin A resulted in hematological improvement but the patient died of disseminated fungal infection. We propose that bone marrow ablation and allografting should be available for these rare cases and suggest that a protocol should be developed with designated transplant centres to aid early referral. © 1997 John Wiley & Sons, Ltd.