The involvement of the gastrointestinal tract in posttransplant lymphoproliferative disease in pediatric liver transplantation.

ABSTRACT

BACKGROUND: Posttransplant lymphoproliferative disease (PTLD) is a serious complication associated with the use of immunosuppression after transplantation. In a retrospective study the clinical features of PTLD located primarily in the gastrointestinal tract were analyzed. METHODS: Three hundred ninety-two consecutive pediatric patients who underwent orthotopic liver transplantation (OLT) during a 13-year period with a survival of more than 6 months were reviewed. Two immunosuppression protocols were used cyclosporin A, or tacrolimus-based primary therapy. Twenty-nine randomly selected liver transplant recipients without PTLD were used for comparison of signs and symptoms of gastrointestinal PTLD. RESULTS: Among the 30 patients identified with PTLD, 9 had gastrointestinal PTLD. The overall incidence density of PTLD was 1.8 per 100 patient-years (30/392). Nine patients (30%) had involvement of the gastrointestinal tract, whereas 7 (23%) had the gastrointestinal tract as the only involved site. When compared with a cohort of liver transplant recipients without PTLD, only gastrointestinal bleeding, weight loss, hypoalbuminemia, and protein-losing enteropathy were signs most likely associated with gastrointestinal PTLD. Hypoalbuminemia was the most sensitive sign of gastrointestinal PTLD. The lower tract (ileum and colon) was the most common site of involvement. CONCLUSIONS: gastrointestinal involvement is common and occurs in 30% of all patients with PTLD. It may be the only affected organ in a subgroup of patients. Hypoalbuminemia, gastrointestinal bleeding, and weight loss are features that are characteristic of gastrointestinal PTLD. Patients with aggressive gastrointestinal signs and symptoms should undergo upper and lower gastrointestinal tract endoscopy with biopsy, to establish the diagnosis.