Posttransplant lymphoproliferative disorder after umbilical cord blood transplantation in children

Jerald Z Gong1, Michael G Bayerl, Linda M Sandhaus

  • 1Department of Pathology, Duke University Medical Center, Durham, NC 27710, USA. gong0001@mc.duke.edu

Insights

Posttransplant lymphoproliferative disorder (PTLD) is a serious Epstein-Barr virus (EBV)-related complication in children after umbilical cord blood transplantation (UCBT). PTLD can originate from the donor or host and monomorphic histology indicates a poor prognosis.

Area of Science:

  • Pediatric Hematology Oncology
  • Transplantation Immunology
  • Infectious Diseases

Background:

  • Umbilical cord blood transplantation (UCBT) is a life-saving procedure for pediatric patients with hematologic malignancies and other conditions.
  • Posttransplant lymphoproliferative disorder (PTLD) is a rare but serious complication following solid organ and hematopoietic stem cell transplantation.
  • The incidence and characteristics of PTLD specifically after UCBT in children are not well-defined.

Observation:

  • This report details 7 pediatric cases of PTLD occurring after UCBT, with a median onset of 4 months post-transplant.
  • Patients presented with advanced disease, often involving visceral organs.
  • Epstein-Barr virus (EBV) was detected in all PTLD cases, with recipients showing prior EBV infection but donors testing negative for EBV DNA.

Findings:

  • PTLD in UCBT recipients can be of host or donor origin, correlating with engraftment status.
  • Monomorphic PTLD histology (5 cases) was associated with a poor response to treatment and rapid mortality.
  • Polymorphic PTLD histology (2 cases) showed a complete response to therapy, with one long-term survivor.

Implications:

  • PTLD following pediatric UCBT is an early, EBV-driven complication requiring prompt recognition and management.
  • Histological subtype (monomorphic vs. polymorphic) is a critical prognostic factor in PTLD after UCBT.
  • Understanding the origin of PTLD (host vs. donor) may inform therapeutic strategies and improve outcomes in pediatric UCBT recipients.