RENAL-ALLOGRAFT INVOLVEMENT BY EPSTEIN-BARR-VIRUS ASSOCIATED POSTTRANSPLANT LYMPHOPROLIFERATIVE DISEASE

This study describes nine cases of post-transplant lymphoproliferative disease (PTLD) presenting as renal allograft dysfunction. Onset of sy mptoms was 34 to 265 days post-transplant, typically (in six of nine c ases) after refractory rejection treated with OKT3. Diagnosis was made by histopathologic examination of needle biopsy (three of nine cases) or allograft nephrectomy (six of nine cases) specimens. Disease was c onfined to the allograft in three patients. The morphology was polymor phic in eight cases and monomorphic in one case. Five cases showed mon otypic kappa or lambda light chain expression. Expansile lymphoid infi ltrates, serpiginous necrosis, nuclear atypia, and presence of Epstein -Barr virus RNA helped to distinguish PTLD from severe rejection. Tubu lar damage and venulitis was common in PTLD lesions, but arterial invo lvement was not prominent. Infiltration of the ureter, hilar adipose t issue, and nerve twigs was frequent in nephrectomy specimens. Reductio n of immunosuppression led to resolution of PTLD in two of three cases diagnosed by needle biopsy, but severe acute rejection led to graft l oss in one case; the third case progressed to fatal multisystem diseas e. Among cases diagnosed at nephrectomy, two of six patients died of d isseminated PTLD and one of six died of sepsis. The five surviving pat ients are alive 41 to 99 months after initial diagnosis without eviden ce of recurrent PTLD.