title: Management of post-transplant lymphoproliferative disorders.
abstract: Post-transplant lymphoproliferative disease (PTLD) is a major complication of hematopoietic stem cell and solid organ transplantation. The incidence of transplantation in childhood has been steadily rising, making PTLD the most common form of lymphoproliferation in childhood. The purpose of this review is to summarize the role of the Epstein-Barr virus (EBV) in the pathophysiology and discuss the management of PTLD.
  
  
  
  More than 90% of pediatric PTLD is EBV-positive. In immunocompetent hosts, the virus is controlled by cytotoxic T-cells, the cells targeted by immunosuppression to avoid graft-versus-host disease and/or organ rejection in transplant patients. The majority of pediatric transplant candidates are EBV-negative prior to transplant increasing the risk of EBV-induced lymphoproliferation upon seroconversion after transplant. Treatment options include reduction of immunosuppression, anti-CD20 monoclonal antibodies, and/or chemotherapy. Advanced understanding of the importance of cellular immunity in controlling lymphoproliferation has led to the development of cellular therapies targeting virus-specific antigens.
  
  
  
  PTLD is the most common form of lymphoproliferation in childhood due to the rising incidence of transplantation. EBV plays a pivotal role in the pathophysiology. Cellular therapies targeting viral antigens may replace chemotherapy in the treatment of PTLD in the near future.
authors.affiliation: aDepartment of Pediatrics, Icahn School of Medicine at Mount Sinai, New York, New York bDepartment of Pediatrics, Children's National Center, Washington, District of Columbia, USA.
authors.forename: Gabriela
authors.initials: G
authors.lastname: Llaurador
authors.forename: Lauren
authors.initials: L
authors.lastname: McLaughlin
authors.forename: Birte
authors.initials: B
authors.lastname: Wistinghausen
mesh_terms: D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000175:diagnosis / Q000209:etiology / Q000628:therapy* / Q000821:virology; D016377:Organ Transplantation; D011183:Postoperative Complications / Q000175:diagnosis / Q000276:immunology / Q000628:therapy* / Q000821:virology