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

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 involvement. Serological data demons trated a reactivation of the EBV infection. Tumor progression with liver in volvement occured during treatment with conventional chemotherapy. Tumor re duction and disappearence of all masses was seen after starting high-dose s equential chemotherapy, followed by an autologous peripheral blood progenit or transplantation. LMP-I could be amplified in the tumor material by PCR t echnology, but no LMP-1 expression could be found in the few malignant B-ce lls with Reed-Sternberg morphology. Sequence analysis of the carboxy termin al of the LMP-1 region revealed the naturally occuring 30 bp deletion varia nt of the LMP-1 with multiple point mutations within the NF kb region. Sinc e LMP-1 was not expressed in the malignant tumor cells, no evidence could b e found, that EBV participated in the tumorigenesis of this case.