DISSEMINATED, MULTICLONAL EPSTEIN-BARR VIRUS-ASSOCIATED LYMPHOPROLIFERATIVE DISEASE IN A PATIENT WITH HEMATOLOGICAL AND IMMUNOLOGICAL ANOMALIES - MOLECULAR ANALYSIS CORRELATES WITH MORPHOLOGICAL APPEARANCE

We report a case of a 21-year-old woman with hematopoietic, immunologi cal, and congenital dysmorphic abnormalities, who died following rapid ly progressive, disseminated Epstein-Barr virus (EBV)-associated lymph oproliferative disease (LPD). Polymerase chain reaction (PCR) amplific ation of formalin-fixed paraffin-embedded tissue showed differences in the clonality of each separate lymphoproliferative lesion examined, a s determined by immunoglobulin heavy chain (IgH) gene rearrangement. P CR analysis also demonstrated that all lesions contained EBV genome. S ince DNA had been extracted from paraffin blocks, a direct comparison of morphology and clonality could be made in each individual lesion. T he evidence from this study indicates that the monodonal tumors arose de novo in multiple sites and that the polyclonal background observed in some lesions reflected a substantial concomitant inflammatory respo nse.