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

✍ Scribed by George Bird; Darryl Peel; Keith McCarthy; Hugh Williams

• Publisher: John Wiley and Sons
• Year: 1997
• Tongue: English
• Weight: 49 KB
• Volume: 15
• Category: Article
• ISSN: 0278-0232
• DOI: 10.1002/(sici)1099-1069(199702)15:1<47::aid-hon597>3.0.co;2-1

✦ Synopsis

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