Факторы прогноза и результаты терапии первичной системной анапластической крупноклеточной лимфомы

Abstract

The retrospective study of clinical data, risk factors, and results of treatment of a large group of patients with primary systemic anaplastic large-cell lymphoma (ALCL) (n = 42) with the Т-/О phenotype is presented. Primary systemic ALCL occurred in young persons aged less than 30 years. A group of patients with its poor course (with complete remission (CR) being achieved) showed a trend for the prevalence of the signs that were a part of the standard (International Prognostic Index — IPI) factors and ones of poor prognosis, which were additionally analyzed by the authors. Significant differences in a group of patients with a good prognosis (with CR being achieved) were obtained only in the following indices: ALK protein expression (substantiating the further division and study of a homogenous group of patients), the stage of the disease, evaluation of the patients’ general condition by the ECOG scale, and the presence of B-symptoms.According to the data available in the literature, ALK-positive ALCL has a better prognosis than ALK-negative ALCL. The results given in this communication additionally confirm and extend these observations. There is evidence that, by using the IPI and the baseline prevalence of the disease, one may predict an outcome in the homogenous group of patients without CR being achieved after first-line therapy. Analysis of the findings has established no clinical risk factors associated with the baseline site of a tumor process, which influence long-term results, but this matter should be studied in further prospective investigations, by keeping in mind the location zones characteristic of ALCL.

Keywords

• anaplastic large-cell lymphoma
• poor prognostic factors
• long-term results of therapy