Post‐transplant lymphoproliferative disease in children

Abstract

Epstein-Barr virus (EBV)-driven post-transplant lymphoproliferative disease (PTLD) is an important cause of morbidity and mortality following transplantation, and it occurs more frequently in children than in adults. Of 22 (5%) children at our institution who developed tissue-proven PTLD 1-60 months (mean 16.5 months) following organ transplant, 11 died: nine of these 22 patients developed PTLD between 1989 and 1993, and seven (78%) died; the remaining 13 developed PTLD between 1994 and 1998, and four (31%) died (p = 0.08). All nine patients who developed PTLD < 6 months after transplant died, but 11 of 13 patients who manifested disease > or = 6 months after transplant survived (p = 0.0002). Ten of 11 (91%) survivors, but only two of eight (25%) children who died, had serologic evidence of EBV infection at the time of PTLD diagnosis (p = 0.04). EBV seroconversion identified patients at risk for developing PTLD, but also characterized patients with sufficient immune function to survive EBV-related lymphoid proliferation. In situ hybridization for EBER1 mRNA was diagnostically helpful because it detected EBV in tissue sections of all 20 patients with B-cell PTLD, including those with negative serology.

Early onset, EBV− PTLD in pediatric liver-small bowel transplantation recipients: a spectrum of plasma cell neoplasms with favorable prognosis

“…1,5,6 Polyclonal plasma cell hyperplasia is a relatively common presentation of early PTLD, whereas frank plasmacytomas and multiple myelomas are rarely described in pediatric SOT recipients. 1 We describe a unique group of pediatric patients with EBV Ϫ plasma cell PTLDs with morphology ranging from atypical plasma cell hyperplasia to plasmacytoma-like lesions.…”

“…2,3 Children have a higher incidence of PTLDs compared with adult recipients. 4,5 Most PTLDs in the pediatric age group are EBV ϩ after the development of primary EBV infection in the posttransplantation period. 1 EBV seronegativity at the time of transplantation is one of the main risk factors for PTLD development, and a large number of children undergoing transplantation are EBV naive.…”

Management of post-transplant Epstein-Barr virus-related lymphoproliferative disease in solid organ and hematopoietic stem cell recipients

“…Mortality due to EBV-related PTLD is also high, ranging from 25% to 48%, and is more frequent in patients who develop PTLD in the fi rst six months post-transplant 8 . The main risk factors are: I) having transplant receptors susceptible to EBV; II) being less than 5 years of age (risk decreases with age), III) being in the fi rst 12 months post-transplant; and IV) a high level of immunosuppressive Review Article treatment, mainly through the administration of antithymocyte globulin (ATG) or muromonab-CD3 (Orthoclone OKT3).…”

Immunotherapy for Epstein-Barr Virus-Associated Cancers in Children

“…The type and degree of immunosuppression varies with the type of organ transplanted. While the overall incidence is estimated at 1%-15%, PTLD is more common after lung and small bowel transplants than after renal, heart, and liver transplants because of the more intense suppression required and the lymphoid tissue present in the graft [37][38][39][40]. Up to 50% of EBV-naïve individuals receiving a transplant from an EBV-seropositive donor experience a symptomatic primary infection, which is then frequently followed by PTLD.…”