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.
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
Background: According to the WHO, T/NK-cell large granular lymphocytic leukemia (T/NK-LGL) comprises 2 – 5 % of all T/NK tumors. In addition, T/NK-LGL is a very heterogeneous disease. T/NK-LGL has been divided along 2 basic types: T-cell and NKcell leukemias. We found further differences among these two basic types. Among our patients with T/NK-LGL, there were 5 immunophenotypic variants with differences in severity of their disease and other clinical parameters. Materials and Methods: Thirty five patients were diagnosed with T/NK-LGL from 01.1998 to 01.2008 (13 men and 22 women) in the age from 18 to 71 years (median age 58 years). The median survival has not been achieved. The median follow up is 72 months. Clinical common features: lymphocytosis in blood and bone marrow, cytopenia (80%), splenomegaly, rheumatoid arthritis and B-symptoms. Unusual features: lymphadenopathy, vasculitis and neuropathy. Diagnostic methods: histology, cytology and flow two-color cytometry with monoclonal antibodies (“Becton Dickinson Immunocytometry Systems” USA and “Dako” Denmark). Clonality was defined by a PCR- on T-cell receptor gene rearrangement. Results: Twenty six patients: 18 women and 8 men were accurately classified as «T/NK - cellular leukemia - LGL» according to WHO classification. The remaining 9 cases had rare immunophenotypes. All (35) patients were subdivided into 5 groups by immunophenotypic (IFT) features of leukemic lymphocytes. 1st group (4 women) had the following IFT of the leukemic lymphocytes: CD2+CD3−CD56+/−CD16+. In the 2d group, 9 patients (5 men and 4 women) had IFT of CD2+CD3+CD8+CD16+CD56+/−. There were 13 patients (3 men and 10 women) in the 3d group. Variant of IFT with leukemic T-cells was CD3+CD8+CD16−CD56−. There were 5 patients (2 men and 3 women) in the 4th group. 20–60 % peripheral blood lymphocytes simultaneous expressed CD8 and CD4 (double – positive T-cells). Others had 40–20% lymphocytes expressing only CD8+ marker. In the 5th group, there were 4 patients (2 men and 2 women). The pathologic T-cells were detected only on the bone marrow aspirate. There had heavy bone marrow aplasia or predominantly red cell aplasia. IFT of lymphocytes in the bone marrow was aberrant with presence of some markers of T-cells, including, CD8, absence of CD16 and CD56. The first three groups appeared stable and did not require chemotherapy for prolonged time (11 of 26 patients, overall 42%). A median of follow-up without treatment is 70 months. The patients of 4th and 5th group required immediate intervention and were treated with chemotherapy. Conclusions: Immunohistochemical characteristics of T-cells separate T/NK-LGL patients with stable disease from those that present with cytopenias and require immediate intervention. The molecular characteristics of these cells will be further defined.
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