Posttransplant Lymphoproliferative Disorder in Adult Lung Transplant Recipients: A Report on Twenty-Seven Patients.

Posttransplant lymphoproliferative disorder (PTLD) is a serious complication of organ transplantation. We report a case series on the diagnosis, treatment and outcome of PTLD in lung transplant recipients. From 1991 to 2006, 27 (5%) of the 502 lung transplant recipients at our institution developed PTLD. The median age at transplant was 51.5 years (range 21–65 years) and the median time from transplant to PTLD diagnosis was 33.7 months (range 1–174 months). Most cases had an elevated LDH (86%) and were EBV-positive (85%). Of the cases tested (n=14), 64% were monoclonal. PTLD was most commonly diagnosed in the lung (56%) and gastrointestinal tract (26%). Three patients were diagnosed at autopsy, while the remaining 24 received therapy. Eight patients were initially treated with reduced immunosuppression (RI) alone, but only 3 (38%) obtained a complete response (CR). Rituxan (R) was used alone (n=3) or in combination with RI (n=3) as initial therapy in six patients, four of whom obtained a CR. Five patients utilized RI +/− surgical resection as a primary treatment modality, all of whom achieved a CR. However, all of them relapsed and required further treatment. Of these five patients initially treated with RI +/− surgical resection, a CR was reestablished in 3 using rituxan alone (1/3), combined rituxan and reduced immunosuppression (1/3) and radiation treatment alone (1/3). The remaining five patients from the 24 patients receiving induction therapy for PTLD were given other treatments. With an average follow-up of 28 months (range 1-130 months), 81% of patients have died. However, only 18% of deaths were directly related to progressive PTLD. The remaining 17 deaths were due to: infection unrelated to PTLD treatment (15%), multi-organ failure (7%), graft failure not due to RI (30%), stroke (4%), and renal failure (4%). Most deaths can be attributed to disease specific and posttransplant complications rather than PTLD. Although an unfortunate diagnosis, PTLD is responsive to therapy, particularly reduced immunosuppression and rituximab.