Classification and Survival of 179 Patients with T- and NK-Cell Lymphomas at the Federal Research Hematology Center of Russian Academy of Medical Sciences: A Retrospective Study.

Abstract

Introduction: According to the new WHO classification for Non-Hodgkin’s lymphomas developed in 2001, the diagnosis of many lymphoma patients has to be reevaluated. The existing information on therapeutic options for patients with these rare malignancies prior to development of WHO classification has been unreliable. The T-cell malignancies are associated with poor outcome, and it is important to identify patients that could benefit from novel intensive therapies. Materials and Methods: We conducted retrospective analysis of patients with T- and NK-cell malignancies treated from 2000–2006 at the Federal Research Hematology Center of Russian Academy of Medical Sciences. The purpose of this study was to determine the frequency, clincopathological features, and outcomes of patients in predominantly Slavic population (European part of Russia). Results: Because these malignancies present in a variety of nosological forms and their response to standard therapies is poor, multiple therapeutic regimens were employed as expected. Information on 151 patients with complete clinical and pathologic information that allowed appropriate diagnosis according to the WHO criteria was available for the analysis. Analysis of 188 patients required changes from the initial diagnosis after additional laboratory investigation: 25 cases (13.3%) previously considered to be T-cell lymphomas were excluded from the analysis, mostly because of an inadequate histopathological material. Similar to previously reported data, a significant number of T- and NK- cell lymphomas (12.4%) were excluded as compared to B-cell (3.1%) lymphomas, which emphasizes the difficulties in diagnosis of mature T- and NK-cell lymphomas. Twelve out of 163 patients with verified T-cell lymphomas after correction of diagnosis were treated and observed in other countries or regions, and were also excluded from the final analysis. The only difference in 151 remaining patients was an increased incidence of T-cell malignancies with mediastinal involvement, with overall survival similar to those reported elsewhere. Final analysis of 28 additional cases in 2006 for a total of 179 patients will be presented. Conclusions: Our survey of patients with T-cell malignancies represents a systematic analysis of these rare malignancies and establishes the communality of clinical features and response to therapies. Kaplan-Mayer Survival of all TCL Kaplan-Mayer Survival of all TCL