Annals of Oncology 9:849-855, 1998
© 1998 European Society for Medical Oncology
research-article |
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
1Hospital Clinic of Barcelona Madrid
2Institut Catala d'Oncologia Madrid
3Residencia ‘Vail d'Hebron’ Madrid
4Hospital ‘Ramón y Cajal’ Madrid
5Fundación ‘Jimenez Diaz’ Madrid
6Hospital Clinico Salamanca
7Hospital Universitari ‘Germans Trias i Pujol’ Valencia, Spain
8Hospital de Sant Pau, Barcelona for the GELC (Grup d'Estudi dels Limfomes a Catalunya) Valencia, Spain
9Hospital Clinico Universitario Valencia, Spain
Correspondence to: E. Montserrat, MD, Hematology Department, Hospital Clinic of Barcelona, Villarroel 170, 08036 Barcelona, Spain E-mail: alopezg{at}medicina.ub.es
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.
Patients and 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-1-positive (ALCL), 30 cases (17%); angioimmunoblastic T cell, 22 cases (13%); angiocentric, 14 cases (8%); intestinal T cell, 12 cases (7%), and hepatosplenic 
5 T cell, one case (0.6%). As compared to the other types, ALCL presented more frequently in ambulatory performance status, without extra-nodal involvement, in early stage, normal serum pß2-micro-globulin (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–t8). 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.
peripheral T-cell lymphomas, prognosis
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