title: Posttransplantation lymphoproliferative disorder in liver recipients: characteristics, management, and outcome.
abstract: Posttransplantation lymphoproliferative disorder (PTLD) is a well-recognized complication of organ transplantation. The aim of this study, performed over 9 years, was to examine the histopathological findings, clinical course, and outcome of patients who, having undergone orthotopic liver transplantation (OLT), developed PTLD. The sample included 7 adult liver allograft recipients (1.7%), 4 men and 3 women, with a mean age of 53 years (range, 40 to 61 years) who developed PTLD 1 to 36 months post-OLT (mean, 6 months). Four patients received either antithymocyte globulin as primary immunosuppression or OKT3 for steroid-resistant cellular rejection. Four patients had localized hepatic tumor with or without regional lymph node involvement, 2 patients had extralymphoreticular disease (head of pancreas and chest wall), and 1 patient had spleen and lymph node involvement. All tumors were B-cell lymphomas; three polymorphic and four monomorphic. Clonality was assessed by immunostaining for kappa and lambda and gene rearrangement. Monoclonality was found in 4 patients and polyclonality in 2 (1 of whom progressed to monoclonality); in 2 patients, clonality could not be determined. Immunohistochemistry findings for the presence of the Epstein-Barr virus (EBV)-determined nuclear antigen and the latent membrane protein 1 were noted in lymphoma tissue in 6 patients. Immunosuppressive therapy was decreased in all patients. Polyclonal tumors were treated with acyclovir (1 patient is in complete remission and 1 patient died), and monoclonal tumors with systemic chemotherapy (2 patients are in complete remission and 2 patients died). One patient was treated with monoclonal antibodies (CD20) but failed to respond, and 1 patient was treated with excision and is in complete remission. The mortality rate was 43%; for the remainder, median survival is 21 months (range, 10 to 42 months). We conclude that PTLD may re-present early after OLT. EBV has a special role in the pathogenesis, combined with immunosuppressive therapy. The outcome is poor, and new therapeutic approaches are needed.
authors.affiliation: Liver Institute and Department of Transplantation, Rabin Medical Center, Beilinson Campus, Petah Tiqva, and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Z
authors.initials: Z
authors.lastname: Ben-Ari
authors.forename: P
authors.initials: P
authors.lastname: Amlot
authors.forename: S R
authors.initials: SR
authors.lastname: Lachmanan
authors.forename: R
authors.initials: R
authors.lastname: Tur-Kaspa
authors.forename: K
authors.initials: K
authors.lastname: Rolles
authors.forename: A K
authors.initials: AK
authors.lastname: Burroughs
mesh_terms: D000328:Adult; D005260:Female; D006566:Herpesviridae Infections / Q000209:etiology / Q000401:mortality / Q000628:therapy; D004854:Herpesvirus 4, Human / Q000302:isolation & purification; D006801:Humans; D007165:Immunosuppression Therapy / Q000009:adverse effects; D007166:Immunosuppressive Agents / Q000009:adverse effects; D016031:Liver Transplantation; D016393:Lymphoma, B-Cell / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D008232:Lymphoproliferative Disorders / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D015996:Survival Rate; D016896:Treatment Outcome; D014412:Tumor Virus Infections / Q000209:etiology / Q000401:mortality / Q000628:therapy
chemical_list: D007166:Immunosuppressive Agents