title: Central nervous system lymphoproliferative disorder in pediatric kidney transplant recipients.
abstract: Post-transplant lymphoproliferative disorder (PTLD) is a complication of transplantation resulting from impaired immune surveillance because of pharmacologic immunosuppression. We present two cases of central nervous system (CNS) PTLD in children on calcineurin-inhibitor free immunosuppression with dramatically different presentations and outcomes. One patient had brain and spinal cord lymphoma with a rapid and fatal course. The other patient had brain and ocular PTLD that responded to multimodal therapy with reduction of immunosuppression, high-dose steroids, and rituximab given in a dose-escalation protocol. This protocol may have enhanced the penetration of rituximab into the CNS. We review the literature on CNS and ocular PTLD and elaborate on the treatments available for both diseases.
authors.affiliation: Division of Nephrology, Children's Hospital, Boston, MA 02115, USA. avram.traum@childrens.harvard.edu
authors.forename: Avram Z
authors.initials: AZ
authors.lastname: Traum
authors.forename: Nancy M
authors.initials: NM
authors.lastname: Rodig
authors.forename: Monika E
authors.initials: ME
authors.lastname: Pilichowska
authors.forename: Michael J G
authors.initials: MJ
authors.lastname: Somers
mesh_terms: D000293:Adolescent; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D002493:Central Nervous System Diseases / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D002675:Child, Preschool; D004305:Dose-Response Relationship, Drug; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016030:Kidney Transplantation / Q000009:adverse effects*; D008223:Lymphoma / Q000209:etiology / Q000473:pathology; D008232:Lymphoproliferative Disorders / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D008297:Male; D011859:Radiography; D012196:Review Literature as Topic; D000069283:Rituximab; D013256:Steroids / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D013256:Steroids; D000069283:Rituximab