title: Plasmablastic posttransplant lymphoma: cytogenetic aberrations and lack of Epstein-Barr virus association linked with poor outcome in the prospective German Posttransplant Lymphoproliferative Disorder Registry.
abstract: BACKGROUND
  Plasmablastic posttransplant lymphoma is a rare subtype of monomorphic B-cell posttransplant lymphoproliferative disorder (PTLD). There is little published clinical data to guide treatment.
  
  
  METHODS
  The German prospective PTLD registry D2006-2012 records baseline features, treatment, and outcome of rare PTLD subtypes in adults after solid organ transplantation. Treatment is at the discretion of the local physician. Clinical data on the patients in the registry is collected before, during, and at least 4 weeks, 6 months, 12 and 24 months after treatment.
  
  
  RESULTS
  Eight cases of plasmablastic posttransplant lymphoma were reported to the registry until 2011. The majority occurred as late PTLD in male heart transplant recipients. Extranodal manifestations were common in stage I and in stage IV disease. Histological Epstein-Barr virus (EBV) association was confirmed in five of eight cases. MYC/IGH rearrangement was present in two of six patients examined. Although five of eight patients died from early disease progression, we observed that long-term survival can be achieved in localized (2/3) and in disseminated disease (1/5) by immunosuppression reduction (IR) followed by immediate systemic chemotherapy. However, all patients with cytogenetic aberrations and patients with non-EBV-associated PTLD were refractory to IR and to chemotherapy. Chemotherapy parallel to IR was associated with a high rate of infectious complications.
  
  
  CONCLUSIONS
  In this series, IR and local therapy were not sufficient to treat plasmablastic posttransplant lymphoma even in localized disease whereas IR and systemic chemotherapy (CHOP-21) could achieve lasting complete remissions. Cytogenetic aberrations and lack of EBV association were linked with poor outcome.
authors.affiliation: Department of Internal Medicine II: Hematology and Oncology, University Medical Centre Schleswig-Holstein, Campus Kiel, Kiel, Germany.
authors.forename: Heiner
authors.initials: H
authors.lastname: Zimmermann
authors.forename: Ilske
authors.initials: I
authors.lastname: Oschlies
authors.forename: Susanne
authors.initials: S
authors.lastname: Fink
authors.forename: Christiane
authors.initials: C
authors.lastname: Pott
authors.forename: Hans H
authors.initials: HH
authors.lastname: Neumayer
authors.forename: Hans
authors.initials: H
authors.lastname: Lehmkuhl
authors.forename: Ingeborg A
authors.initials: IA
authors.lastname: Hauser
authors.forename: Martin
authors.initials: M
authors.lastname: Dreyling
authors.forename: Michael
authors.initials: M
authors.lastname: Kneba
authors.forename: Barbara
authors.initials: B
authors.lastname: Gärtner
authors.forename: Ioannis
authors.initials: I
authors.lastname: Anagnostopoulos
authors.forename: Hanno
authors.initials: H
authors.lastname: Riess
authors.forename: Wolfram
authors.initials: W
authors.lastname: Klapper
authors.forename: Ralf U
authors.initials: RU
authors.lastname: Trappe
mesh_terms: D000328:Adult; D000368:Aged; D002869:Chromosome Aberrations; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D005260:Female; D015321:Gene Rearrangement; D005858:Germany; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D016393:Lymphoma, B-Cell / Q000209:etiology* / Q000235:genetics / Q000401:mortality / Q000628:therapy / Q000821:virology; D008232:Lymphoproliferative Disorders / Q000209:etiology* / Q000235:genetics / Q000401:mortality / Q000628:therapy / Q000821:virology; D008297:Male; D008875:Middle Aged; D016377:Organ Transplantation / Q000009:adverse effects* / Q000401:mortality; D011446:Prospective Studies; D012042:Registries; D018570:Risk Assessment; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome