Successful eradication of rituximab-refractory EBV-related post- transplant lymphoproliferative disorder after second haplo-identical allogeneic hematopoietic stem cell transplantation for very severe aplastic anemia – a case report

Abstract

EBV-related post-transplant lymphoproliferative disorder (PTLD) is a potentially fatal disorder arising after solid organ or hematopoietic stem cell transplant (HSCT). The principle of management includes reduction of immune suppression, rituximab, and chemotherapy. We presented a female patient of very severe aplastic anemia (VSAA) complicated with initial graft dysfunction and then graft failure 2 months after haplo-identical allogeneic HSCT, with no response to salvage cyclosporine plus eltrombopag. EBV-related PTLD occurred 6 months after first haplo-transplant as shown by high EBV PCR. The patient initially responded to 6 shots of weekly rituximab, and then progression to ultrahigh level of EBV DNAemia was observed with the presence of intra-hepatic PTLD that was refractory to rituximab. This patient subsequently underwent second haplo-identical allogeneic transplant from the same donor with megadose of stem cell number plus alkylating agent containing RIC regimen. The results were successful hematopoietic engraftment on day +15, clearance of EBV DNAemia in one month, and thereafter eradication of intra-hepatic EBV-PTLD. In conclusion, EBV-related PTLD refractory to rituximab alone is still sensitive to rituximab plus chemotherapy preparative regimen during subsequent allogeneic HSCT, and it is still feasible to perform allo-transplant for transplant-eligible patients despite the presence of rituximab-refractory EBV DNAemia and PTLD.