title: Experience with 274 cardiac transplant recipients with posttransplant lymphoproliferative disorder: a report from the Israel Penn International Transplant Tumor Registry.
abstract: BACKGROUND
  Posttransplant lymphoproliferative disorder (PTLD) is a life-threatening complication that occurs in a small but significant minority of solid organ transplant recipients. Published experiences with PTLD in cardiac transplant recipients are limited to relatively small single-center reports.
  
  
  METHODS
  This report presents experience with 274 cases of PTLD in cardiac transplant recipients reported to the Israel Penn International Transplant Tumor Registry (IPITTR).
  
  
  RESULTS
  PTLD carried an ominous prognosis: Kaplan Meier survival after PTLD diagnosis was 45%, 33%, 30%, and 13%, respectively, at 1, 3, 5, and 10 years. Common causes of death included: PTLD, cardiovascular collapse, and infection; all occurred at a median of less than 6 months. Risk of death from cardiovascular collapse secondary to immunosuppression withdrawal was substantial (28%), indicating that a fine balance exists between death from PTLD and from sudden cardiac death due to acute rejection. PTLD therapy in the majority of patients consisted of combination therapy (49%). Survival in patients receiving immunosuppression minimization (ISM) alone was 32%, with ISM plus other therapy was 27%, and with other therapies not containing ISM was 11% (P < 0.01).
  
  
  CONCLUSIONS
  PTLD in cardiac transplant recipients is associated with low long-term survival rates. Analysis of PTLD therapies and outcomes suggest that immunosuppression minimization, when applied, improves survival. However, risk of sudden death may mitigate the positive effect of ISM. This observation has important implications for ISM in PTLD therapy in cardiac transplant recipients. Carefully designed prospective studies are needed to evaluate the positive and negative effects of ISM in cardiac transplant recipients with PTLD.
authors.affiliation: Department of Pharmacy, New York Presbyterian Hospital-New York Weill Cornell Center, New York, NY, USA.
authors.forename: Meredith J
authors.initials: MJ
authors.lastname: Aull
authors.forename: Joseph F
authors.initials: JF
authors.lastname: Buell
authors.forename: Jennifer
authors.initials: J
authors.lastname: Trofe
authors.forename: M Roy
authors.initials: MR
authors.lastname: First
authors.forename: Rita R
authors.initials: RR
authors.lastname: Alloway
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Hanaway
authors.forename: Lynne
authors.initials: L
authors.lastname: Wagoner
authors.forename: Thomas G
authors.initials: TG
authors.lastname: Gross
authors.forename: Thomas
authors.initials: T
authors.lastname: Beebe
authors.forename: E Steve
authors.initials: ES
authors.lastname: Woodle
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D016027:Heart Transplantation / Q000009:adverse effects* / Q000401:mortality; D006801:Humans; D007165:Immunosuppression Therapy; D008232:Lymphoproliferative Disorders / Q000209:etiology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D012042:Registries