Epstein-Barr virus induced virus-associated hemophagocytic syndrome and monoclonal TCR-beta rearrangement: a case report

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.