Esther Ramos, Ane Miren Andrés, Eva Martínez-Ojinaga, Jesús María Sarría, Manuel Molina, José Luis Encinas, Francisco Hernández, Nuria Leal, Manuel Gámez, Manuel López, Gerardo Prieto

Intestinal Rehabilitation Unit, Hospital Infantil La Paz, Madrid, Spain

Introduction: Post-transplant lymphoproliferative disorders (PTLD) are a well-recognised and potentially fatal complication after intestinal transplantation. It is a heterogeneous group of disorders with a wide spectrum of pathologic and clinical manifestations. We analyzed the incidence, clinical features and outcome in a 60 intestinal transplantation series.

Patients and Methods: Between October 1999 and December 2010, 51 paediatric patients received 60 intestinal transplants. Graft types included: Isolated small bowel (ISB) (n= 22; 36.6%), liver and small bowel (LSB) (n=20; 33.3%), and multivisceral (MV) (n=18; 30%). Median age at transplant was 3.1 years (range: 0.5-18). Based on immunosuppressive regimen patients were categorized into three groups: I (n=42) received basiliximab, tacrolimus and steroids; II (n=11) thymoglobulin and tacrolimus and III (n=7) alemtuzumab and tacrolimus. Epstein-Barr virus (EBV) replication was serially measured. All PTLD cases were biopsied to establish a histopathologic diagnosis.

Results: The incidence of PTLD was 13.3% (8/60). The median age of patients was 2.5 years (range: 1.5-8) and 5 (62.5%) were girls. Median onset of PTLD after transplant was 2.7 months (range: 0.5-21), within first postoperative year in 6 (75%) patients. The incidence by immunosuppression groups were: group I 14.3%, group II 9.1%, and group III 14.3% and by graft type: ISB 18.2%, LSB 10%, and MV 11.1%. Histologic types were polymorphic B cell EBV+ in 2 (25%), monomorphic B cell lymphoma EBV+ in 4 (50%), peripheral T cell lymphoma EBV- in 1 (12.5%), and histiocytic sarcoma EBV- in 1 (12.5%). Clinical presentation: fever (100%), lymphadenopathy, tonsillar hypertrophy, and graft failure. The patient survival rate was 62.5% (5/8).

Conclusions: PTLD is a common and frequently early complication after intestinal transplant. The immunosuppressive regimen and graft type were not associated with the risk of developing PTLD. Most cases are EBV-related B cell tumours. All patients developed fever. PTLD-related mortality was high.