*Purpose: Post-transplant Lymphoproliferative Disorders (PTLD) after solid organ transplantation is a complication of chronic immunosuppression (IS) and is generally associated with Epstein Barr virus (EBV) infection. It presents with high morbidity and mortality, especially in children, affecting 1-5% of kidney transplants. The main risk factors are: low age and EBV seronegative pre-transplant status. Conventional treatment strategies are reduction of IS, anti-CD20 therapy (rituximab) and chemotherapy in some cases.

*Methods: Case series of PTLD in a pediatric cohort population of 103 patients who received a renal transplant between August 2007 and April 2018.

*Results: Five patients were diagnosed with PTLD (incidence 4.8%), all males, median age at diagnosis of 4 years (3 -11 years), 80% (4/5) received deceased donor kidney. Induction IS was basiliximab in 80% (4/5) of the cases and thymoglobulin in 20% (1/5). Maintenance IS was tacrolimus (Tac), mycophenolate mofetil (MMF) and prednisone in all cases. The pre transplant recipients EBV status was negative in 60% (3/5) of patients. The median time to the PTLD diagnosis was 6 months (2-10 months) post transplantation with the following symptoms: suppurative tonsillitis (2/5), fever (2/5), gastrointestinal tract ulcers (1/5), pain and abdominal mass (1/5), neutropenia (1/5) and worsening of kidney function (3/5). Whole blood quantitative EBV PCR was positive in 60% (3/5) of patients (543, 1.584 and 6.214 cp/mL) and in situ hybridization demonstrating EBV positivity in tissues was present in 80% of patients. Tacrolimus and mycophenolate were withdrawn in all cases. 4/5 patients received 4-6 doses of rituximab, 2/5 patients received cyclophosphamide and 1/5 received vincristine. mTOR inhibitor was initiated one month after treatment with subsequent reintroduction of MMF and/or Tac in 80% (4/5) of patients. Two acute rejection episodes occurred 3 and 52 months after PTLD treatment (humoral and cellular rejection respectively). During a median follow-up of 52 months (7-94m), no patient relapsed, median current estimated glomerular filtration rate (Schwartz equation) was 103 ml/min/1.73m² (range 73-111), with 100% graft and patient survival.

*Conclusions: The incidence and the low age of PTLD manifestation are in accordance with the literature. The PTLD presentation was clinically diverse and early after transplantation with a low copy count. The quantitative EBV PCR did not help the early detection of the neoplasia in our case series. During follow-up, two patients evolved with rejection, with 100% graft and patient survival.

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