title: Management of posttransplant lymphoproliferative disease in pediatric liver transplant recipients receiving primary tacrolimus (FK506) therapy.
abstract: BACKGROUND
  Posttransplant lymphoproliferative disease (PTLD) after pediatric liver transplantation has been associated with high mortality rates.
  
  
  METHODS
  The present study examined 282 consecutive pediatric liver transplant recipients from October 1989 to June 1996 who received primary tacrolimus immunosuppression. The aim was to determine the incidence of PTLD, management strategies, and patient outcome.
  
  
  RESULTS
  The incidence of PTLD was 13% (361282) with a mean age of 5.5+/-0.7 years (range 0.6 to 15) at diagnosis. The average time from transplantation to PTLD was 10.1+/-2.1 months. Initial treatment of PTLD consisted of reduction (3 patients) or discontinuation (33 patients) of tacrolimus and initiation of antiviral therapy (intravenous ganciclovir, 14 patients; intravenous acyclovir, 22 patients; or both, 5 patients). Alpha-interferon was used in four patients (two successfully). One patient also received gamma-interferon, chemotherapy, and radiation for a central nervous system lesion. Chemotherapy was also used in one patient with Burkitt's, whereas one patient with a pulmonary lesion received additional radiation therapy. Three patients received supportive surgery for gastrointestinal involvement, and one patient had a splenectomy for hemolysis. Overall mortality was 22% (8/36) with 5 (14%) PTLD-related deaths (disseminated disease, 4 patients; bowel perforation, 1 patient). Of 31 survivors, 23 had acute rejection at a median time of 24 days after PTLD, with 2 patients developing chronic rejection. One patient required retransplantation. Present immunosuppression consists of tacrolimus monotherapy in 14 patients, tacrolimus/prednisone in 8 patients, and none in 6 patients.
  
  
  CONCLUSIONS
  In summary, PTLD can be successfully treated with reduction of immunosuppression and administration of antiviral agents in most patients. The management of rejection after PTLD requires reassessment of disease status and judicious reintroduction of immunosuppression therapy.
authors.affiliation: Department of Transplant Surgery, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh, Pennsylvania 15213, USA.
authors.forename: T V
authors.initials: TV
authors.lastname: Cacciarelli
authors.forename: M
authors.initials: M
authors.lastname: Green
authors.forename: R
authors.initials: R
authors.lastname: Jaffe
authors.forename: G V
authors.initials: GV
authors.lastname: Mazariegos
authors.forename: A
authors.initials: A
authors.lastname: Jain
authors.forename: J J
authors.initials: JJ
authors.lastname: Fung
authors.forename: J
authors.initials: J
authors.lastname: Reyes
mesh_terms: D000293:Adolescent; D000998:Antiviral Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000188:drug therapy; D006084:Graft Rejection / Q000453:epidemiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D015994:Incidence; D007223:Infant; D007422:Intestines / Q000601:surgery; D016031:Liver Transplantation; D008232:Lymphoproliferative Disorders / Q000453:epidemiology / Q000401:mortality / Q000628:therapy* / Q000821:virology; D011183:Postoperative Complications / Q000628:therapy*; D011241:Prednisone / Q000627:therapeutic use; D012086:Reoperation; D013156:Splenectomy; D016019:Survival Analysis; D016559:Tacrolimus / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D000998:Antiviral Agents; D007166:Immunosuppressive Agents; D011241:Prednisone; D016559:Tacrolimus