cells were scattered around cardiac muscle, suggesting that persistent EBV infection was involved in the degeneration of specialized cardiac muscle, Atrioventricular block has also been reported in EBV myocarditis. 9 These findings suggest that prolonged EBV infection could lead to the degeneration of specialized cardiac muscle over a long period.Allogeneic HSCT has been reported to offer a good prognosis for those with CAEBV. 2,3 A national survey, however, performed in Japan found a high risk of HSCT-related mortality. 5 Recently, successful treatment of CAEBV infection using reducedintensity stem cell transplantation (RIST) with fludarabine and melphalan with or without anti-thymocyte globulin (ATG) has been reported, 10,11 which could control CAEBV by reconstituting host immunity against EBV. Regimen-related toxicity is expected to be more effectively alleviated using a reduced intensity conditioning regimen for transplantation than by conventional myeloablative conditioning in those with an impaired residual function of organs. In contrast, it is well known that the risk of EBV-related complications after transplantation might increase with the additional use of ATG in non-myeloablative conditioning. 12 In the current case we adapted fludarabine-based, reducedintensity conditioning with ATG for transplantation, because it was predicted that the residual cardiac function might be partly impaired by coronary giant aneurysms. The additional use of ATG, however, in fludarabine-based conditioning might have been involved in the development of hemophagocytic syndrome during preconditioning for transplantation, although it was uncertain whether the serum sickness induced by the use of ATG was associated with the degeneration of cardiac muscle. To answer the many remaining questions, such as the best transplantation method for CAEBV, the optimal timing of transplantation, and the most effective conditioning regimen, a multicenter-based clinical trial is needed.
References1 Cohen JI. Epstein-Barr virus infection. N. Engl. J. Med. 2000; 343: 481-92. 2 Okano M. Overview and problematic standpoints of severe chronic active Epstein-Barr virus infection syndrome. Crit. Rev. Oncol. Hematol. 2002; 44: 273-82.