AGGRESSIVE TREATMENT FOR POSTCARDIAC TRANSPLANT LYMPHOPROLIFERATION

Posttransplant lymphoproliferative disorder (PTLD) is a frequently fat al complication of organ transplantation, occurring in 2% to 6% of car diac recipients. Treatment remains poorly defined, Reduction in immuno suppression is effective in a proportion of cases, but mortality on th e order of 80% is reported for patients requiring chemotherapy, The re ason for such poor outcomes is unclear, but may be partly caused by th e concomitant use of immunosuppressives. Nineteen consecutive cardiac recipients with PTLD were studied retrospectively in terms of clinical features and outcome. Patients were managed according to a uniform tr eatment approach. Initial therapy was a trial of reduced immunosuppres sion with concomitant acyclovir followed, if unsuccessful, by aggressi ve combination chemotherapy. The regimen used was predominantly ProMAC E-CytaBOM. Six patients with phenotypically polyclonal PTLD presented less than 6 months after transplantation (median 6 weeks). Only 1 of 4 (25%) treated patients responded to reduced immunosuppression; the re mainder died of multiorgan failure. Thirteen patients presented with p henotypically monoclonal disease greater than or equal to 6 months aft er transplantation. In 8 of 12 (75%) treated patients initial therapy was reduction in immunosuppression. None achieved complete remission ( CR) and 2 experienced fatal rejection. Two patients achieved durable s urgical CR. The remaining 8 patients received chemotherapy; 2 of 8 (25 %) died during treatment, 6 of 8 (75%) achieved CR. None have relapsed , at a median duration of follow-up of 38 months. Neutropenic sepsis a nd subclinical doxorubicin cardiotoxicity at a mean cumulative dose of 63 mg/m(2) were the principal toxicities. Our data indicate that aggr essive chemotherapy is both feasible and effective in phenotypically m onoclonal PTLD refractory to reduced immunosuppression. ProMACE-CytaBO M is well suited to cardiac recipients, minimizing doxorubicin exposur e and obviating the need for concurrent immunosuppressives. (C) 1995 b y The American Society of Hematology.