Epstein-Barr virus related hemophagocytic syndrome in a T-cell rich B-cell lymphoma

We report the case of a 30-year-old woman who presented with an EBV related hemophagocytic syndrome. After a few months she developed a T-cell rich B-cell non-Hodgkin's lymphoma with liver involvment. Serological data demonstrated a reactivation of the EBV infection. Tumor progression with liver involvement occured during treatment with conventional chemotherapy. Tumor reduction and disappearence of all masses was seen after starting high-dose sequential chemotherapy, followed by an autologous peripheral blood progenitor transplantation. LMP-I could be amplified in the tumor material by PCR technology, but no LMP-1 expression could be found in the few malignant B-cells with Reed Sternberg morphology. Sequence analysis of the carboxy terminal of the LMP-I region revealed the naturally occuring 30 bp deletion variant of the LMP-I with multiple point mutations within the NF kb region. Since LMP-I was not expressed in the malignant tumor cells, no evidence could be found, that EBV participated in the tumorigenesis of this case.