title: Development of a biologically distinct EBV-related lymphoproliferative disorder following autologous bone marrow transplantation for an EBV-negative post-renal allograft Burkitt's lymphoma.
abstract: Post-transplant lymphoproliferative disorder (PTLD) is a known complication of both solid organ transplantation and allogeneic bone marrow transplantation (BMT) but is rarely seen following autologous BMT. We report the case of a 45 year-old female who developed Burkitt's lymphoma eight years after a renal allograft. This PTLD was found to have lambda light chain restriction, contained del(8)(q24) and add(14)(q32), and was negative for EBV on immunohistochemical and DNA-based PCR analyses. Immunoglobulin heavy chain (IgH) PCR studies revealed a prominent clonal rearrangement. She responded to intravenous cyclophosphamide and proceeded to high-dose chemoradiotherapy and mafosfamide-purged autologous BMT. Thirty-nine days post-BMT she presented with cough and fever and developed hepatic dysfunction; abnormal lymphoplasmacytoid cells were noted in the peripheral blood. Investigations revealed kappa light chain restriction, an oligoclonal IgH rearrangement, a normal karyotype and PCR studies for EBV were positive, consistent with a clinically and biologically distinct PTLD. She initially improved following discontinuation of immunosuppression, but then deteriorated abruptly and died 58 days post-BMT. It is likely that the two separate episodes of PTLD in this patient, one of which was atypical, arose as a result of both the chronic use of cyclosporine and the impairment of cell-mediated immunity associated with autologous BMT. The sequence of events in this patient should contribute to a better understanding of late-onset, EBV-negative PTLD.
authors.affiliation: The Leukemia and Bone Marrow Transplantation Program of British Columbia: Division of Hematology, British Columbia Cancer Agency, Vancouver, Canada.
authors.forename: C A
authors.initials: CA
authors.lastname: Lohrisch
authors.forename: T J
authors.initials: TJ
authors.lastname: Nevill
authors.forename: M J
authors.initials: MJ
authors.lastname: Barnett
authors.forename: D E
authors.initials: DE
authors.lastname: Hogge
authors.forename: J M
authors.initials: JM
authors.lastname: Connors
authors.forename: P A
authors.initials: PA
authors.lastname: Keown
authors.forename: R D
authors.initials: RD
authors.lastname: Gascoyne
mesh_terms: D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D002051:Burkitt Lymphoma / Q000601:surgery*; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000209:etiology*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000009:adverse effects; D016030:Kidney Transplantation / Q000009:adverse effects; D008232:Lymphoproliferative Disorders / Q000188:drug therapy / Q000209:etiology / Q000821:virology*; D008875:Middle Aged; D016609:Neoplasms, Second Primary / Q000209:etiology / Q000821:virology; D014182:Transplantation, Autologous; D014184:Transplantation, Homologous / Q000009:adverse effects
chemical_list: D007166:Immunosuppressive Agents