title: Post-transplant Burkitt lymphoma is a more aggressive and distinct form of post-transplant lymphoproliferative disorder.
abstract: BACKGROUND
  Although the literature reports a low incidence of Burkitt lymphoma (BL) as a post-transplant lymphoproliferative disorder (PTLD), this entity appears to be different from other monomorphic PTLDs (M-PTLDs), both in its aggressive clinical presentation and its distinct pathologic profile.
  
  
  METHODS
  Patients with BL, diagnosed in the post-transplant setting, (patients aged ≤ 18 years) were retrieved from the pathology archives at Children's Hospital of Pittsburgh of the University of Pittsburgh Medical Center from 1982 to 2010. Clinical outcomes were obtained along with pathologic review.
  
  
  RESULTS
  Twelve patients with pediatric BL in the post-transplant setting (9 boys, 3 girls) were retrieved. The patients displayed a monomorphic population of small to intermediate-sized, noncleaved, lymphoid elements with a "starry-sky" pattern. The immunophenotype for patients available to the study was CD20+ (n = 9/10), CD10+ (n = 8/8), bcl-6+ (n = 11/11), with a near 100% Ki-67/MIB-1 proliferation index (n = 7/7), and negative for TdT (n = 7/7). Most pretransplant Epstein-Barr virus titers were negative (n = 8/10), with post-transplant titers positive in all tested patients (n = 11), and with positive Epstein-Barr-encoded RNA in situ hybridization in most cases (n = 9/11). The median time from transplantation to diagnosis was 52 months (range, 6-107 months). Nine patients were currently alive after immediate antineoplastic chemotherapy, with median disease-free time of 93 months from diagnosis (range, 2-199 months).
  
  
  CONCLUSIONS
  BL-PTLD had a higher Epstein-Barr virus incidence compared with sporadic and immunodeficiency-associated BL and represented a distinct monomorphic PTLD. Although some M-PTLDs can be managed less aggressively with decreased immunosuppression alone, immediate lymphoma-specific chemotherapy was associated with a favorable outcome and was strongly recommended.
authors: Department of Pediatric Pathology, Children's Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA. picarsicj@upmc.edu
authors: Jennifer
authors: J
authors: Picarsic
authors: Ronald
authors: R
authors: Jaffe
authors: George
authors: G
authors: Mazariegos
authors: Steve A
authors: SA
authors: Webber
authors: Demetrius
authors: D
authors: Ellis
authors: Michael D
authors: MD
authors: Green
authors: Miguel
authors: M
authors: Reyes-Múgica
mesh_terms: D000293:Adolescent; D002051:Burkitt Lymphoma / Q000209:etiology* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D004279:DNA, Viral / Q000235:genetics; D020031:Epstein-Barr Virus Infections / Q000209:etiology* / Q000473:pathology; D005260:Female; D004854:Herpesvirus 4, Human / Q000235:genetics; D006801:Humans; D016130:Immunophenotyping; D017404:In Situ Hybridization, Fluorescence; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008232:Lymphoproliferative Disorders / Q000150:complications* / Q000473:pathology / Q000628:therapy; D008297:Male; D011183:Postoperative Complications; D033581:Stem Cell Transplantation / Q000009:adverse effects*; D016896:Treatment Outcome
chemical_list: D004279:DNA, Viral