Post-transplant lymphoproliferative disorders are not associated with IgG4 sclerosing disease

H. P. Cathro*, G. C. Bullock, H. Bonatti, Z. Meriden, S. Cook, N. Aguilera

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review


Background: Although the majority of post-transplant lymphoproliferative disorder (PTLD) cases are associated with Epstein-Barr virus (EBV), 20-42% of cases are EBV negative (EBV-N). The antigenic stimulus that drives EBV-N PTLD is unknown, but is likely heterogeneous. A common feature of PTLD, regardless of EBV status, is an abnormal polytypic lymphoplasmacytic infiltrate. Immunglobulin-G4 (IgG4) syndrome is also characterized by a polytypic lymphoplasmacytic infiltrate with a predominance of IgG4-positive (IgG4-P) plasma cells. Methods: We investigated the possibility of an association between EBV-N PTLD and IgG4 syndrome. Of 33 evaluated PTLD cases, 9 (27%) were EBV-N. EBV-N PTLD cases showed longer transplantation-to-diagnosis times than EBV-positive cases. Results: A single patient had a preceding benign duodenal biopsy with focally prominent IgG4-P plasma cells; however, no clinical data supported IgG4 syndrome, precluding an association between IgG4 syndrome and subsequent EBV-N PTLD in this patient. Conclusion: As none of 29 evaluable cases of PTLD (including all 9 EBV-N cases) were associated with an increase in IgG4-P plasma cells, IgG4 syndrome does not appear to play a role in the etiology of EBV-N PTLD. The significance of these findings and the current understanding of the etiology of EBV-N PTLD are discussed.

Original languageEnglish (US)
Pages (from-to)897-903
Number of pages7
JournalTransplant Infectious Disease
Issue number6
StatePublished - Dec 1 2014


  • EBV-negative
  • Epstein-Barr virus
  • IgG4
  • Post-transplantation lymphoproliferative disorder

ASJC Scopus subject areas

  • Transplantation
  • Infectious Diseases


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