title: Burkitt post-transplantation lymphoma in adult solid organ transplant recipients: sequential immunochemotherapy with rituximab (R) followed by cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) or R-CHOP is safe and effective in an analysis of 8 patients.
abstract: BACKGROUND
  Burkitt lymphoma post-transplantation lymphoproliferative disorder (Burkitt-PTLD) is a rare form of monomorphic B-cell PTLD for which no standard treatment has been established. Currently, the treatment of Burkitt lymphoma outside the post-transplantation setting involves high doses of alkylating agents, frequent dosing, and intrathecal and/or systemic central nervous system prophylaxis. In PTLD, however, such protocols are associated with considerable toxicity and mortality.
  
  
  METHODS
  The authors present a retrospective series of 8 adult patients with Burkitt-PTLD. Six patients were reported to the prospective German PTLD registry or were enrolled in the PTLD-1 trial, and 2 patients had received treatment before 2000, thus allowing for comparison with the pre-rituximab era.
  
  
  RESULTS
  Seven of the 8 patients were men. The median age at presentation was 38 years, and the median time since transplantation was 5.7 years. Five of 8 patients had histologically established, Epstein-Barr virus-associated disease, and 7 of 7 patients were positive for a MYC translocation. Five of 8 patients received sequential immunochemotherapy (4 courses of rituximab [R] followed by 4 cycles of cyclophosphamide, doxorubicin, vincristine, and prednisolone [CHOP] or R plus CHOP [R-CHOP]). In this group, 5 of 5 patients reached complete remission (CR), and their overall survival (OS) was significantly longer (P = .008) compared with the OS for 2 of 8 patients who received first-line CHOP and did not respond. One of 8 patients (who had stage IV disease with meningiosis) received combination therapy (cyclophosphamide pretreatment, rituximab, intrathecal chemotherapy, whole-brain irradiation, and radioimmunotherapy) and reached CR. Overall, 6 of 8 patients reached CR; and, after a median follow-up of 4.7 years (range, 1.7-4.8 years), the median OS was 36.7 months. There was no treatment-related mortality under first-line therapy.
  
  
  CONCLUSIONS
  In the largest adult case series in Burkitt-PTLD to date, sequential immunochemotherapy with rituximab followed by standard CHOP or R-CHOP was a both safe and effective treatment.
authors.affiliation: Department of Internal Medicine II, University Medical Center Schleswig-Holstein, Campus Kiel, Kiel, Germany.
authors.forename: Heiner
authors.initials: H
authors.lastname: Zimmermann
authors.forename: Petra
authors.initials: P
authors.lastname: Reinke
authors.forename: Ruth
authors.initials: R
authors.lastname: Neuhaus
authors.forename: Hans
authors.initials: H
authors.lastname: Lehmkuhl
authors.forename: Stephan
authors.initials: S
authors.lastname: Oertel
authors.forename: Johannes
authors.initials: J
authors.lastname: Atta
authors.forename: Manfred
authors.initials: M
authors.lastname: Planker
authors.forename: Barbara
authors.initials: B
authors.lastname: Gärtner
authors.forename: Dido
authors.initials: D
authors.lastname: Lenze
authors.forename: Ioannis
authors.initials: I
authors.lastname: Anagnostopoulos
authors.forename: Hanno
authors.initials: H
authors.lastname: Riess
authors.forename: Ralf U
authors.initials: RU
authors.lastname: Trappe
mesh_terms: D000328:Adult; D000368:Aged; D000465:Algorithms; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use*; D014408:Biomarkers, Tumor / Q000032:analysis; D002051:Burkitt Lymphoma / Q000737:chemistry / Q000188:drug therapy* / Q000276:immunology* / Q000473:pathology / Q000821:virology; D016371:Cranial Irradiation; D003520:Cyclophosphamide / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D020031:Epstein-Barr Virus Infections / Q000150:complications; D005260:Female; D005500:Follow-Up Studies; D005858:Germany; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D017404:In Situ Hybridization, Fluorescence; D007278:Injections, Spinal; D008297:Male; D008875:Middle Aged; D016377:Organ Transplantation; D011241:Prednisone / Q000008:administration & dosage; D012042:Registries; D012074:Remission Induction; D012189:Retrospective Studies; D000069283:Rituximab; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D014408:Biomarkers, Tumor; D007155:Immunologic Factors; D000069283:Rituximab; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone