title: Peripheral T-cell lymphomas: initial features, natural history, and prognostic factors in a series of 174 patients diagnosed according to the R.E.A.L. Classification.
abstract: BACKGROUND
  Peripheral T-cell lymphomas (PTCL) account for about 10% of all lymphomas in Western countries. The aim of the present study is to analyze the initial characteristics and prognostic factors in a large series of PTCL patients.
  
  
  METHODS
  174 patients (105 male/69 female; median age 61 years) were diagnosed with PTCL according to the R.E.A.L. Classification in nine Spanish institutions between 1985 and 1996. Cutaneous lymphomas and T-cell chronic lymphocytic/prolymphocytic leukemia were excluded from the study. Univariate and multivariate analyses were used to assess the prognostic value of the main initial variables.
  
  
  RESULTS
  The distribution according to histology subgroup was: PTCL unspecified, 95 cases (54.4%); anaplastic large-cell Ki-l-positive (ALCL), 30 cases (17%); angioimmunoblastic T cell, 22 cases (13%); angiocentric, 14 cases (8%); intestinal T cell, 12 cases (7%), and hepatosplenic gamma delta T cell, one case (0.6%). As compared to the other types, ALCL presented more frequently in ambulatory performance status, without extranodal involvement, in early stage, normal serum beta 2-microglobulin (B2M) level and low-risk international prognostic index (IPI). Most patients were treated with adriamycin-containing regimens. The overall CR rate was 49% (69% for ALCL vs. 45% for other PTCL; P < 0.02). The risk of relapse was 48% at four years. Median survival of the series was 22 months (65 months for ALCL vs. 20 months for other PTCL; P = 0.03), with a four-year probability of survival of 38% (95% confidence intervals (95% CI): 28-48). In the univariate analysis, in addition to the histology, older age, poor performance status, presence of B-symptoms, extranodal involvement, bone marrow infiltration, advanced Ann Arbor stage, high serum LDH, high serum B2M, and intermediate- or high-risk IPI were related to poor survival. In the multivariate analysis the histologic subgroup (ALCL vs. other PTCL) (P = 0.02; response rate (RR): 4.3), the presence of B-symptoms (P = 0.02, RR: 2.2), and the IPI (low vs. high) (P = 0.04, RR: 2) maintained independent predictive value. When the analysis was restricted to the unspecified subtype, only IPI had independent prognostic value (P = 0.003; RR: 3.5).
  
  
  CONCLUSIONS
  PTCL have adverse prognostic features at diagnosis, respond poorly to therapy and have short survival, with no sustained remission. ALCL constitutes a subgroup which responds better to therapy and has a longer survival.
authors.affiliation: Hospital Clinic of Barcelona, Spain. alopezg@medicina.ub.es
authors.forename: A
authors.initials: A
authors.lastname: López-Guillermo
authors.forename: J
authors.initials: J
authors.lastname: Cid
authors.forename: A
authors.initials: A
authors.lastname: Salar
authors.forename: A
authors.initials: A
authors.lastname: López
authors.forename: C
authors.initials: C
authors.lastname: Montalbán
authors.forename: J M
authors.initials: JM
authors.lastname: Castrillo
authors.forename: M
authors.initials: M
authors.lastname: González
authors.forename: J M
authors.initials: JM
authors.lastname: Ribera
authors.forename: S
authors.initials: S
authors.lastname: Brunet
authors.forename: J
authors.initials: J
authors.lastname: García-Conde
authors.forename: A
authors.initials: A
authors.lastname: Fernández de Sevilla
authors.forename: F
authors.initials: F
authors.lastname: Bosch
authors.forename: E
authors.initials: E
authors.lastname: Montserrat
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000970:Antineoplastic Agents / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000032:analysis*; D005260:Female; D006801:Humans; D016411:Lymphoma, T-Cell, Peripheral / Q000145:classification / Q000473:pathology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D011379:Prognosis; D012307:Risk Factors; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D014408:Biomarkers, Tumor