RESUMO
UNLABELLED: Primary central nervous system post-transplant lymphoproliferative disease (CNS PTLD) is a serious complication after solid organ transplantation that has not received much attention so far. However, it could become a more frequent problem with the introduction of new biological agents. METHODS: We identified five cases with CNS PTLD in our center who were transplanted between 1986 and 2007, three men and two women, with a mean age of 55.9 years (range 42-74). Three patients had received only kidney transplant and two patients had received a kidney-pancreas transplant. RESULTS: The mean time from first symptoms until diagnosis was 3.5 months (2-6). One patient was diagnosed post-mortem in autopsy. The mean time from transplantation to onset of neurological symptoms was 73.8 months (31-144). The initial clinical manifestation was heterogeneous: all five cases showed headache, four cases presented with gait disturbance, one with dysarthria and two with a confusional state. Epstein-Barr virus (EBV) immunoglobulin (Ig)G serology was positive in four out of five cases; in situ hybridization for EBV in brain biopsy samples was positive in three cases, negative in one and not available in one. In four patients, EBV polymerase chain reaction (PCR) was positive in cerebrospinal fluid (CSF). After diagnosis, overall immunosuppressive load was lowered in all patients (n = 4). Three patients died at 8-104 weeks (mean 40 weeks) after diagnosis and one patient is still alive 20 months after diagnosis. CONCLUSIONS: CNS PTLD is a complication difficult to diagnose, frequently diagnosed too late and often refractory to treatment. A more aggressive screening might be necessary in patients even with mild CNS symptoms.