Low-dose chemotherapy for Epstein-Barr virus-positive post-transplantation lymphoproliferative disease in children after solid organ transplantation

Thomas G. Gross, John C. Bucuvalas, Julie R. Park, Timothy C. Greiner, Steven H. Hinrich, Stuart S. Kaufman, Alan N. Langnas, Ruth A. McDonald, Frederick C. Ryckman, Byers W. Shaw, Debra L. Sudan, James C. Lynch

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120 Scopus citations

Abstract

Purpose: To evaluate the efficacy of a low-dose chemotherapy regimen in children with Epstein-Barr virus (EBV) -positive, post-transplantation lymphoproliferative disease (PTLD) after organ transplantation who have experienced failure with front-line therapy for PTLD. Patients and Methods: Eligible patients received cyclophosphamide (600 mg/m2 intravenous for 1 day) and prednisone (2 mg/kg orally for 5 days) every 3 weeks for six cycles. Results: Thirty-six patients treated on study were assessable for analyses. Front-line therapies for PTLD before study entry included immune suppression reduction or withdrawal (n = 36), antiviral therapy (n = 33), surgical resection (n = 8), rituximab (n = 2), and interferon alfa (n = 1). Reasons for failure of front-line therapy included progressive disease (PD; n = 33) and persistent disease with concurrent allograft rejection (n =3). Thirty patients (83%) had stage III to IV disease, 92% had extranodal disease, and 75% had > three sites of disease. The overall response rate was 83% (75% complete response + 8% partial response). The relapse rate was 19%, with only one of five relapsed patients alive and disease-free. Four patients presented with fulminant, disseminated PTLD; only one of these four patients achieved a response, and all four died of PD. Two patients died of treatment-related toxicity. Three patients (8%) experienced allograft loss, but two of the three patients are alive and disease-free after a second transplantation. The 2-year overall, relapse-free, and failure-free (without PTLD and with functioning original allograft) survival rates were 73%, 69%, and 67%, respectively. Conclusion: This low-dose chemotherapy regimen is effective for children with EBV-positive, nonfulminant PTLD who have experienced treatment failure with frontline therapy, and this study represents the largest series of PTLD patients treated prospectively with a uniform chemotherapy regimen.

Original languageEnglish (US)
Pages (from-to)6481-6488
Number of pages8
JournalJournal of Clinical Oncology
Volume23
Issue number27
DOIs
Publication statusPublished - Sep 20 2005

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ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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