Clinical characteristics, treatment outcome and survival of 36 adult patients with primary anaplastic large cell lymphoma

Background and Objective. Although in recent years anaplastic large-cell ly mphoma (ALCL) has emerged as a distinct clinlco-pathological entity, a gold standard for treatment has still not been defined. Goals of our histologic , phenotypic and clinical study were to present clinical findings, treatmen t outcome and survival rates of a small, but highly homogeneously treat ed, series of patients. Design and Methods. From April 1991, 36 newly diagnosed adult patients with systemic ALCL CD30(+), entered a prospective non-randomized trial in one o f the institutions participating in a GISL (Gruppo Italiano per lo studio d ei Linfomi) study and were treated with a MOPP/EBV/CAD hybrid scheme. Chemo therapy (CHT) was administered every 28 days, for a total of 6 cycles. Afte r CHT, 19 patients received radiation therapy (RT) to the site of previousl y involved fields. Kaplan and Meier and log-rank tests were used for statis tical analysis. Results. The overall complete remission rate was 78%, the partial remission rate was 6%. The overall survival rate at 74 months was 69%. No statistica lly significant differences in response or survival rates were noted compar ing ALCL-HL and -CT subgroups, T+ Null- and B- subtypes, or ALCL-HL and -CT , with different phenotypes. In the analysis of patients with T+ Null pheno type treated with CHT+RT in comparison with B-ALCL patients who had the sam e treatment, we observed statistically significant differences in the survi val rate (p=0.048). No prognostic factors predictive of response or surviva l were identified. Interpretation and Conclusions. Our results show that using MOPP/ABV/CAD th e results, in terms of remission rate and survival, are similar to those ob tained with 3(rd) generation CHT regimens. The diagnosis of T and Null ALCL is the mast important prognostic factor, because it Is associated with a v ery good survival, even in patients with a high prognostic index. Finally, we believe that longer follow-ups are needed to evaluate long-term survival and toxicity with different treatments. (C)1999, Ferrata Storti Foundation .