Atypical Presentations of EBV Reactivation and EBV-Associated PTLD Post-HSCT: Implication for Routine EBV Surveillance after Allogeneic HSCT?.

Allogeneic hematopoietic stem cell transplantation (HSCT), an effective treatment for leukemia and other disorders, produces profound immune deficiency in the early period after transplantation. Viral reactivations such as Epstein-Barr virus (EBV) are a major cause of morbidity and mortality after HSCT. EBV-associated posttransplant lymphoproliferative disease (PTLD) is a life-threating and often fatal complication occurring after allogeneic HSCT with a cumulative incidence of 1% at 10 years. Most cases occur during the first year after transplantation with the incidence highest at 2–3 months after HSCT. Typically PTLD presents as a disseminated disease with fever, lymph node enlargement, respiratory disease and feature of hepatitis. The common lymphadenopathy usually leads to diagnosis, with confirmation on viral tests. Here we retrospectively evaluate the patients at our institution since January 2002 to date (07/2005) who were allogeneic transplanted. Of the total 236 patients who were transplanted during this time period eight patients (3.4 %) developed a significant EBV reactivation with treatment need. Of the eight patients six had a fulminant course. Of these five patients died (EBV reactivation/PTLD is at least probable cause of death). Since at least two of the patients with the fulminant course presented atypically with fever as initial symptom and without other manifestations of PTLD like lymph node enlargement, these cases underline the need of an immediate pre-emptive therapy without any time delay for further diagnostic procedures e. g. diagnostic lymphadenectomy. While at present there is no recommendation of the EBMT for routine surveillance after HSCT, our data indicate the need for a standardized surveillance strategy.