title: BCL6 expression correlates with monomorphic histology in children with posttransplantation lymphoproliferative disease.
abstract: Posttransplantation lymphoproliferative disease (PTLD) is a complication of organ transplantation with high mortality. Predicting response to first-line therapy, reduction of immune suppression, is difficult because of the heterogeneity of lesions and disease behavior. We sought to determine if BCL6 protein expression in PTLD cells is associated with poor outcome. In a cohort of 25 children with PTLD, 9 of the patients' tumor specimens were positive for BCL6 protein expression. Eight of 13 monomorphic lesions were BCL6 positive, compared with 1 of 11 evaluable polymorphic lesions (P=0.01). Only 1 of the patients with BCL6 expression responded to reduced immune suppression (P=0.19). Recipients of heart transplants who developed PTLD had reduced overall survival rates compared with recipients of other organ transplants who developed PTLD (P=0.04).
authors.affiliation: Department of Pediatrics, Center for Cancer and Blood Disorders, The Children's Hospital and University of Colorado Denver, Aurora, CO, USA.
authors.forename: Christopher C
authors.initials: CC
authors.lastname: Porter
authors.forename: Xiayuan
authors.initials: X
authors.lastname: Liang
authors.forename: Jane
authors.initials: J
authors.lastname: Gralla
authors.forename: Loris
authors.initials: L
authors.lastname: McGavran
authors.forename: Edythe A
authors.initials: EA
authors.lastname: Albano
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D004268:DNA-Binding Proteins / Q000032:analysis*; D005260:Female; D006801:Humans; D007165:Immunosuppression Therapy; D007223:Infant; D008232:Lymphoproliferative Disorders / Q000175:diagnosis / Q000209:etiology*; D008297:Male; D016377:Organ Transplantation / Q000009:adverse effects* / Q000401:mortality; D011379:Prognosis; D051560:Proto-Oncogene Proteins c-bcl-6; D015996:Survival Rate
chemical_list: C084103:BCL6 protein, human; D004268:DNA-Binding Proteins; D051560:Proto-Oncogene Proteins c-bcl-6
references.citation: Nature. 2004 Dec 2;432(7017):635-9
references.pmid: 15577913
references.citation: Blood. 2003 Feb 15;101(4):1220-35
references.pmid: 12393483
references.citation: Crit Rev Oncol Hematol. 2002 Jan;41(1):1-9
references.pmid: 11796228
references.citation: Recent Results Cancer Res. 2002;159:96-103
references.pmid: 11785850
references.citation: J Pediatr Hematol Oncol. 2001 Jan;23(1):14-8
references.pmid: 11196263
references.citation: Cancer. 1998 May 15;82(10):1978-87
references.pmid: 9587133
references.citation: Blood. 2006 Jun 1;107(11):4207-13
references.pmid: 16449523
references.citation: J Clin Oncol. 1999 Dec;17(12):3835-49
references.pmid: 10577857
references.citation: Pediatr Transplant. 2001 Aug;5(4):250-7
references.pmid: 11472603
references.citation: J Pediatr Hematol Oncol. 2007 Feb;29(2):112-6
references.pmid: 17279008
references.citation: Blood. 2003 Aug 15;102(4):1443-8
references.pmid: 12738680
references.citation: Leukemia. 2002 Feb;16(2):268-75
references.pmid: 11840294
references.citation: Proc Natl Acad Sci U S A. 2004 Sep 28;101(39):14198-203
references.pmid: 15375218
references.citation: Blood. 1995 Jan 15;85(2):552-65
references.pmid: 7812011
references.citation: Transplantation. 1999 Nov 27;68(10):1517-25
references.pmid: 10589949