Post-transplantation lymphoproliferative disorder in pediatric kidney-transplant recipients - a national study.

Institute of Nephrology, Schneider Children's Medical Center of Israel, Petach Tikva, Israel.
Pediatric Transplantation (Impact Factor: 1.63). 06/2012; 16(6):619-26. DOI: 10.1111/j.1399-3046.2012.01731.x
Source: PubMed

ABSTRACT PTLD is the most common malignancy in pediatric kidney-transplant recipients. We examined the prevalence, clinical features, and outcome of PTLD in Israel. Twelve (4.4%) of 272 pediatric (<19 yr) kidney-transplant recipients retrieved from a search of the NIKTR for 1991-2008 had acquired PTLD at a median of 3.2 yr post-transplantation. PTLD-affected patients were younger at transplantation (4.2 vs. 12.5 yr, p = 0.02), had a higher rate of OKT3 therapy for acute rejection (25% vs. 4%, p = 0.015), and 5/12 were EBV-seropositive at transplantation. Graft dysfunction was the presenting sign in six (50%). PTLD was predominantly abdominal (83%) and B-cell type (67%); T-cell PTLD occurred exclusively in EBV-seropositive patients. Treatment consisted of immunosuppression cessation (6/12, 50%), antiviral agents (7/12, 58%), anti-CD20 monoclonal antibodies (4/12, 33%), and chemotherapy (6/12, 50%). Survival was 100% in the EBV-naïve patients and 40% in the EBV-seropositive patients. Graft loss occurred in three of eight survivors (37.5%). PTLD-associated mortality risk was older age: 11.2 vs. 3.4 yr, longer dialysis: 15 vs. 6.5 months, T-cell type disease (75%), later PTLD onset: 6.35 vs. 1.9 yr post-transplantation and era of transplantation (43% mortality before vs. 20% after 2001). Pretransplantation EBV-seronegative status might confer a survival benefit with early detected PTLD. EBV-seropositive patients are at risk for aggressive late-onset lethal PTLD.

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    ABSTRACT: Abstract Objectives: The aim of this study was to detect the frequency, time of occurrence, management and outcome of Epstein-Barr virus (EBV) infection and related complications in pediatric renal transplant recipients. Methods: Pediatric renal allograft recipients transplanted between August 1994 and December 2011 at our hospital was evaluated retrospectively. The patients were divided into two groups; Groups 1 and 2 were composed of patients transplanted before and after November 2007, respectively, when plasma EBV DNA levels were periodically measured. Results: The study included 166 children, 89 (53.6%) boys, with a mean age of 12.2 ± 3.8 years. Prior to transplantation, 144 patients (86.7%) were EBV seropositive. Within a median follow-up period of 36 months, 11 of 22 seronegative children (50%) developed primary EBV infection. EBV reactivation was observed in 23 of 144 children (15.9%). Two patients with primary infection developed post-transplant lymphoproliferative disorder, one of whom died. Elevated serum creatinine levels or graft loss were not observed in any patient with EBV reactivation. Conclusions: EBV DNA monitoring by PCR in high-risk pediatric renal transplant recipients will provide early diagnosis and treatment of EBV infections.
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    ABSTRACT: Patients after solid organ transplantation (SOT) carry a substantially increased risk to develop malignant lymphomas. This is in part due to the immunosuppression required to maintain the function of the organ graft. Depending on the transplanted organ, up to 15% of pediatric transplant recipients acquire posttransplant lymphoproliferative disease (PTLD), and eventually 20% of those succumb to the disease. Early diagnosis of PTLD is often hampered by the unspecific symptoms and the difficult differential diagnosis, which includes atypical infections as well as graft rejection. Treatment of PTLD is limited by the high vulnerability towards antineoplastic chemotherapy in transplanted children. However, new treatment strategies and especially the introduction of the monoclonal anti-CD20 antibody rituximab have dramatically improved outcomes of PTLD. This review discusses risk factors for the development of PTLD in children, summarizes current approaches to therapy, and gives an outlook on developing new treatment modalities like targeted therapy with virus-specific T cells. Finally, monitoring strategies are evaluated.
    Clinical and Developmental Immunology 09/2013; 2013:814973. DOI:10.1155/2013/814973 · 2.93 Impact Factor
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