title: Post-transplant lymphoproliferative disorders: improved outcome after clinico-pathologically tailored treatment.
abstract: OBJECTIVE
  Clinical and pathologic variability of post-transplant lymphoproliferative disorders (PTLDs), their aggressive behavior and the recognized therapy-related toxicity make management of patients with these disorders difficult. Assessment of first-line treatment and identification of prognostic factors need to be better defined.
  
  
  METHODS
  Data on 40 PTLDs which developed in adult solid organ recipients were analyzed in order to evaluate clinical and pathologic features, response to treatment and prognostic factors. Data were collected retrospectively between 1989 and 1996; since 1997 a prospective study has been activated.
  
  
  RESULTS
  The median time from transplant to PTLD was 56 months. Regarding histologic features, plasmacytic hyperplasia was diagnosed in 5 patients (12.5%), polymorphic lymphoproliferative disorders in 3 (7.5%), malignant lymphoma in 32 (80%). The diagnosis was made at autopsy in eight patients (20%). Late-onset PTLDs (>12 months from transplant) occurred in 33 patients (83%), EBV-negative forms in 12 (31%). Relevant differences have been observed between EBV-positive and EBV-negative forms. Twenty-nine patients completed their scheduled treatment and are evaluable for outcome. The cumulative probability of survival at 1 year is 57% (CI 37.6-73.4) and the median survival time of the entire group has not been reached at 54 months. Clinical stage, performance status, lactate dehydrogenase and number of sites are predictive factors for survival. The International Prognostic Index and the PTLD index are able to identify different risk groups.
  
  
  CONCLUSIONS
  Although rare, PTLDs are a significant cause of mortality in allograft recipients. Therapy tailored on histologic and clinical features of PTLD is feasible and is able to give long-lasting complete responses.
authors.affiliation: Department of Oncology-Haematology, Niguarda Hospital, Piazza Ospedale Maggiore, 3 20162 Milan, Italy. ematologia@ospedale-niguarda.it
authors.forename: Giuliana
authors.initials: G
authors.lastname: Muti
authors.forename: Silvia
authors.initials: S
authors.lastname: Cantoni
authors.forename: Pierluigi
authors.initials: P
authors.lastname: Oreste
authors.forename: Catherine
authors.initials: C
authors.lastname: Klersy
authors.forename: Guido
authors.initials: G
authors.lastname: Gini
authors.forename: Valentina
authors.initials: V
authors.lastname: Rossi
authors.forename: Giovanna
authors.initials: G
authors.lastname: D'Avanzo
authors.forename: Patrizia
authors.initials: P
authors.lastname: Comoli
authors.forename: Fausto
authors.initials: F
authors.lastname: Baldanti
authors.forename: Marco
authors.initials: M
authors.lastname: Montillo
authors.forename: Annamaria
authors.initials: A
authors.lastname: Nosari
authors.forename: Enrica
authors.initials: E
authors.lastname: Morra
mesh_terms: D000328:Adult; D000368:Aged; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D000998:Antiviral Agents / Q000627:therapeutic use; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000188:drug therapy; D005260:Female; D016027:Heart Transplantation / Q000009:adverse effects; D016481:Helicobacter Infections / Q000150:complications / Q000188:drug therapy; D016480:Helicobacter pylori / Q000302:isolation & purification; D004854:Herpesvirus 4, Human / Q000302:isolation & purification; D006801:Humans; D016867:Immunocompromised Host; D007165:Immunosuppression Therapy / Q000009:adverse effects*; D016753:Interleukin-10 / Q000097:blood; D016030:Kidney Transplantation / Q000009:adverse effects; D015993:Life Tables; D016031:Liver Transplantation / Q000009:adverse effects; D016040:Lung Transplantation / Q000009:adverse effects; D008232:Lymphoproliferative Disorders / Q000453:epidemiology / Q000209:etiology* / Q000382:microbiology / Q000628:therapy / Q000821:virology; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications / Q000453:epidemiology / Q000209:etiology* / Q000628:therapy / Q000821:virology; D011379:Prognosis; D016016:Proportional Hazards Models; D012074:Remission Induction; D012189:Retrospective Studies; D012307:Risk Factors; D000069283:Rituximab; D016019:Survival Analysis; D015996:Survival Rate; D013997:Time Factors; D014180:Transplantation / Q000009:adverse effects*; D016896:Treatment Outcome; D019562:Viral Load
chemical_list: D000900:Anti-Bacterial Agents; D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000998:Antiviral Agents; D016753:Interleukin-10; D000069283:Rituximab