title: Post-transplant lymphoproliferative disorders are not associated with IgG4 sclerosing disease.
abstract: 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Pathology, University of Virginia, Charlottesville, Virginia, USA.
authors.forename: H P
authors.initials: HP
authors.lastname: Cathro
authors.forename: G C
authors.initials: GC
authors.lastname: Bullock
authors.forename: H
authors.initials: H
authors.lastname: Bonatti
authors.forename: Z
authors.initials: Z
authors.lastname: Meriden
authors.forename: S
authors.initials: S
authors.lastname: Cook
authors.forename: N
authors.initials: N
authors.lastname: Aguilera
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D020031:Epstein-Barr Virus Infections / Q000150:complications; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000378:metabolism*; D008232:Lymphoproliferative Disorders / Q000175:diagnosis / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D016377:Organ Transplantation / Q000009:adverse effects; D010865:Pilot Projects; D011183:Postoperative Complications; D012189:Retrospective Studies; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000209:etiology / Q000378:metabolism* / Q000473:pathology; D055815:Young Adult
chemical_list: D007074:Immunoglobulin G
keywords: EBV-negative; Epstein-Barr virus; IgG4; post-transplantation lymphoproliferative disorder