Oral Communications 1
7.214 - Improved prognosis and survival In intestinal transplant recipients with post-transplant lymphoproliferative disease: A new look at an old disease
Presenter: Laura, Wozniak, , United States
Authors: Laura J. Wozniak1, Robert S. Venick1,2, Tian Mauer2, Pamela Kempert3, Elizabeth A. Marcus1, Vilayphone Hwang2, Kanela Artavia4, Ronald W. Busuttil2, Sue V. McDiarmid1,2, Douglas G. Farmer2
Improved prognosis and survival In intestinal transplant recipients with post-transplant lymphoproliferative disease: A new look at an old disease
Laura J. Wozniak1, Robert S. Venick1,2, Tian Mauer2, Pamela Kempert3, Elizabeth A. Marcus1, Vilayphone Hwang2, Kanela Artavia4, Ronald W. Busuttil2, Sue V. McDiarmid1,2, Douglas G. Farmer2
1Pediatric Gastroenterology, UCLA Mattel Children's Hospital, Los Angeles, CA, United States; 2Surgery/Liver and Pancreas Transplantation, David Geffen School of Medicine at UCLA, Los Angeles, CA, United States; 3Pediatric Hematology Oncology, David Geffen School of Medicine at UCLA, Los Angeles, CA, United States; 4Care Coordination, UCLA Medical Enterprise, Los Angeles, CA, United States
Introduction: Post-transplant lymphoproliferative disease (PTLD) is an important cause of morbidity and mortality following intestinal transplantation (ITx), especially given the robust immunosuppression required. Our aim was to review a single-center PTLD experience with a focus on outcomes. Methods: An IRB-approved, retrospective review of all ITx between 1991-2012 was performed using a prospectively maintained database. All cases of PTLD were included. PTLD treatment was individualized for each patient but universally included immunosuppression reduction. Relevant medical protocols include: IMMUNOSUPPRESSION: Induction therapy is standard; agents are IL2RA (58%), OKT3/ATG (33%), NONE (9%). Maintenance immunosuppression is TAC, PRED, and MMF. RAPA is used as rescue therapy. Acute cellular rejection (ACR) is treated with PRED or OKT3/ATG. ANTIVIRAL PROPHYLAXIS: Ganciclovir prophylaxis is administered for the first 3-5 yrs post-ITx. Blood EBV quantitative PCR is monitored weekly to monthly. Pre-emptive therapy with IV ganciclovir, CMV immunoglobulin, or rituximab is given for elevated or persistent viremia. Results are reported as median (range). Results: Fourteen (13.5%) of 104 ITx recipients developed 16 PTLD cases during a median follow-up time of 59 (19-131) mos. The majority of patients were males (n=9) who received liver-inclusive allografts (n=10). Four patients (29%) were re-ITx recipients. Median age at PTLD diagnosis was 7.2 (2.6-57) yrs; only two were adults. Diagnosis was made at a median of 20 (1.7-107) mos post-ITx. 5/5 cases diagnosed in the first year post ITx were all EBV+ by immunohistochemical stains (IHC) while only 6/11 (55%) of the late PTLD cases were IHC EBV+. Overall, only 6/16 (38%) were associated with EBV viremia (EBV PCR >100 DNA copies/mL). 11/16 (69%) PTLD cases had a history of ITx ACR. PTLD was initially diagnosed in intestinal allograft (n=6), lymph node (n=5), native colon (n=2), tonsil (n=1), lung (n=1), or bone marrow (n=1). PTLD types were categorized as mixed cell (n=6), B-cell (n=5), or plasma cell (n=5). Overall patient and graft survival are both 86%, with a median survival time following PTLD diagnosis of 25 (1.2-89.3) mos. There were two deaths, with only one attributed to PTLD. Two patients are receiving ongoing therapy for recent early-stage PTLD diagnoses. Conclusion: We report excellent outcomes following PTLD in ITx recipients. Our program has a low incidence (<5%) of early PTLD diagnosed in the first year post-ITx, and early PTLD was not associated with worse survival as has been published. In addition, only 55% of our late PTLD cases were EBV-associated. This may be attributed to our program’s aggressive approach to monitoring, preventing, and treating EBV viremia. Furthermore, all ITx recipients with late non-EBV-associated PTLD are disease-free with 100% patient and graft survival. Only one patient was lost to PTLD.