Clinicopathological Features and Outcomes of T- and NK-Cell Lymphomas in European Russia

Abstract

With the introduction of the revised World Health Organization Classification of Tumors of Hematopoietic and Lymphoid Tissues in 2001, many patients had to be re-evaluated for the correct diagnosis of T- and NK-cell lymphomas. Because some T-cell malignancies are associated with poor outcome, it is important to identify subsets of patients that may benefit from novel or more intensive therapies. The purpose of this study was to determine, for the first time, the relative frequencies, pathological features and outcomes of patients with T- and NK-cell lymphomas in a predominantly Russian Slavic population. We identified 291 patients with a diagnosis of T- and NK-cell malignancies treated at our Center between 2000-2008. In applying the revised WHO classification, we confirmed the diagnosis and had complete clinical follow up and pathological information on 264 cases that were included in the analysis. We found some differences in frequency of several subsets as compared with previously published reports, including younger age of onset and relatively higher incidence of T-LGL in our patients. We also confirm that intensive treatment regimens of advanced stage PTCL and ALK—ALCL led to considerable improvement in response rates, but not in the overall survival.

Clonal Rearrangements and Malignant Clones in Peripheral T-cell Lymphoma

“…only CD2 or CD3. In addition, a small number of PTCL-NOS are γδ-T-lymphocytes lymphomas that cannot be classified as hepatolienal γδ-lymphomas or γδ-variant of large granular lymphocytes leukemia based on clinical and morphological data [ 3 - 6 ]. A study of clonal rearrangements of T-cell receptor genes in PTCL-NOS confirms T-cell clonality in complex diagnostic cases and proves the presence of a tumor [ 7 - 11 ].…”