Posttransplant lymphoproliferative disorder in pediatric patients: Survival rates according to primary sites of occurrence and a proposed clinical categorization

Am J Transplant. 2019 Oct;19(10):2764-2774. doi: 10.1111/ajt.15358. Epub 2019 Apr 22.

Abstract

Posttransplant lymphoproliferative disorder (PTLD) is a devastating complication of organ transplant. In a hospital-based registry, we identified biopsy-proven cases of PTLD among children during a 15-year period and reviewed trends in PTLD rates, the sites of involvement, and the associated survival rates. Cases that were included had at least 1 year of follow-up after the diagnosis of PTLD. We studied 82 patients with first-episode PTLD. Median age at diagnosis was 6.4 years (IQR 3.2-12.3 years). The most frequent PTLD sites were tonsillar/adenoidal (T/A [34%]) and gastrointestinal (32%), followed by miscellaneous (defined as less common sites including central nervous system, kidney, lung, and soft tissue [12%]), lymph node (11%), and multisite (11%). Kaplan-Meier survival curves showed that T/A PTLD was associated with decreased all-cause mortality compared with PTLD at other sites (log-rank 0.004), even after adjustment for histological subtype (P = .047). PTLD-related mortality was also decreased among T/A PTLD (log-rank 0.012) but showed a trend toward significance only after adjustment for histological subtype (P = .09). Among first episodes of PTLD, T/A PTLD was associated with a survival advantage compared with PTLD at other sites, even after adjustment for potential confounders. Based on our observations, we propose a clinical categorization of PTLD according to anatomical site of occurrence.

Keywords: Epstein-Barr Virus (EBV); cancer/malignancy/neoplasia; classification systems; clinical research/practice; hematogenous/leukemia/lymphoma; hematology/oncology; infection and infectious agents - viral; infectious disease; patient survival; pediatrics; posttransplant lymphoproliferative disorder (PTLD).

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Female
  • Follow-Up Studies
  • Humans
  • Lymphoproliferative Disorders / etiology
  • Lymphoproliferative Disorders / mortality*
  • Lymphoproliferative Disorders / pathology
  • Male
  • Organ Transplantation / adverse effects
  • Organ Transplantation / mortality*
  • Postoperative Complications / etiology
  • Postoperative Complications / mortality*
  • Postoperative Complications / pathology
  • Prognosis
  • Registries / statistics & numerical data
  • Risk Factors
  • Survival Rate