Peripheral T-cell lymphoma, unspecified - the analysis of the data from the Czech Lymphoma Study Group (CLSG) registry

Vít Procházka ,Inge Bolomska,Barbora Otavova,David Belada,Zdeněk Král ,Jana Zapletalová ,Marek Trněný ,Jiřina Švecová ,J Přibylová ,Hana Šiffnerová ,Tomáš Kozák ,Markéta Petrová ,Milan Matuška ,Ingrid Vášová ,Michaela Hamouzová ,Robert Pytlík ,Tomáš Papajík ,Katarína Petráková ,Milan Lysý ,Kateřina Kubáčková ,Kateřina Langová

doi:10.5507/bp.2007.019

Abstract

Peripheral T-cell lymphoma, unspecified (PTCL-US) is one of the entities from the infrequent family of nodal mature T-cell lymphomas. The clinical course is aggressive, and despite multiagent chemotherapy, the median survival is about 2 years. Published data are limited to retrospective, mostly single-center studies or reviews and usually include more lymphoma subtypes. To evaluate the current treatment modalities, clinical outcome and prognostic factors in unselected, new diagnosed patients with PTCL-US in the population of the central european region (Czech Republic). Czech Lymphoma Study Group is a national scientific organization which provides an on-line database registry which collects a data about almost all new diagnosed lymphoma patients since year 2000. All diagnostic biopsies were reviewed by a reference pathologist. We analyzed 63 patients with new diagnosis of PTCL-US. The median age was 59 years (25-81), chemotherapy (CHT) was administered in 56 of the 63 patients: anthracyclin-based CHT in 51%, intensive CHT in 21% and non-anthracyclin regimen was applied in 13% of the patients. The overall response rate was 74.4%, (CR in 57.4%). After a median follow-up of 19.6 months, 41% of the patients were in CR, 3.4% in PR or stable disease and 55% of the patients died. The estimated survival probability in 3 years was 36%. Clinical stage (IV) and CR achievement were found to be independent survival predictors in a multivariate analysis. Although the current treatment modalities are mostly ineffective in PTCL-US, appropriate intensive treatment may lead to prolonged remission but not survival.

Highlights

Peripheral T-cell lymphomas (PTCLs) are infrequent types of non-Hodgkin‘s lymphomas (NHLs)
Pearson Chi-Square test and the Fisher‘s Exact Test was used to test the associations of bone marrow involvement, clinical stage, splenic involvement, extranodal involvement, performace status (≥ 2), elevated lactate dehydrogenase level, International progostic index (IPI) score, achievement of complete remission, intensive chemotherapy, relapse and complete response (CR) achievement or exitus probability
We analyzed a data of the 63 patients, baseline clinical parameters are summarized in Tab. 1

Summary

Introduction

Peripheral T-cell lymphomas (PTCLs) are infrequent types of non-Hodgkin‘s lymphomas (NHLs). Published data from the Surveillance, Epidemiology, and End Results (SEER) registries in the USA, which included 114 548 lymphoid neoplasms diagnosed during 1992–2001 show evident increase of T-cell lymphoma incidence (up to 6.4 % per year)(ref.[3]). Published World Health Organization (WHO) Classification of Tumours[4] subdivide T-cell lymphomas into predominantly leukemic, extranodal and nodal types. PTCLs usually presents with disseminated, often extranodal disease and outcome of the patients is much worse than in B-cell counterpart lymphomas[5,6,7]. Peripheral T-cell lymphoma, unspecified (PTCL-US) is one of the entities from the infrequent family of nodal mature T-cell lymphomas. Published data are limited to restrospective, mostly single-center studies or reviews and usually include more lymphoma subtypes

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