Clinical features of peripheral T-cell lymphomas in 78 patients diagnosed according to the Revised European-American lymphoma (REAL) classification

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Abstract

The aim of this study was to analyse the clinical characteristics and prognostic factors of peripheral T-cell lymphomas (PTCLs) according to the Revised European-American Lymphoma (REAL) classification. From 1994 to 1999, 78 patients were diagnosed with PTCLs, excluding cutaneous T-cell lymphomas and T-cell chronic lymphocytic leukaemia. The distribution of the histological subgroups were: PTCL unspecified (PTCL-U), 40%; angiocentric lymphoma, 32%; anaplastic large cell lymphoma (ALCL), 17%; angioimmunoblastic T-cell lymphoma (AILD), 6%; intestinal T-cell lymphoma, 3%; and panniculitic T-cell lymphoma, 3%. Patients with angiocentric lymphoma presented with favourable prognostic factors, whereas those with AILD presented with unfavourable prognostic factors. Most patients were treated with doxorubicin-containing combination chemotherapy (with or without radiation therapy). The overall complete remission rate was 61.2% (95% Confidence Interval (CI): 48.5–72.8%) and the 5-year probability of failure-free survival was 33.5%. Median survival of all patients was 45 months (range 0–64+ months) and the 5-year probability of survival was 36.2%. In the multivariate analysis, only the International Prognosis Index (IPI) was an independent prognostic factor for overall survival (P<0.01). Taken together, the proportion of angiocentric lymphoma in this study was higher than that in the studies of Western countries. PTCL responds poorly to treatment with low survival rates and the IPI is a useful prognostic factor for PTCL.

Introduction

Peripheral T-cell lymphomas (PTCLs) are neoplasia from post-thymic T-cells at different stages of differentiation and are a heterogeneous group of malignancies which present with different morphological patterns, phenotypes and clinical presentations 1, 2, 3, 4, 5, 6, 7, 8. Recent reports suggest that Epstein–Barr virus (EBV) may play an important role in the lymphomagenesis of PTCLs [9]. These tumours have a striking epidemiological distribution with a lower incidence in Western countries than in Asia 2, 3, 4, 5, 6, 7, 8, 13, are common in Japan and Taiwan and are frequently associated with human T-lymphotropic virus (HTLV-1) 8, 21. In Korea, PTCLs including T- or natural killer (NK)-cell lymphomas constitute approximately 25 to 35% of all non-Hodgkin's lymphomas 2, 18, 19.

Cytological features alone are not sufficient to distinguish between PTCL disease entities. Clinical presentation is very important in the classification of PTCLs 4, 11. Although a few studies about the clinical features of PTCLs according to the Revised European-American Lymphoma (REAL) classification have been reported recently [1], the clinical features of each entity are not fully understood because of their rarity and broad cytomorphological spectrum 1, 2, 3, 4, 5, 6, 7, 11. There is substantial disagreement in the literature concerning the prognostic parameters to be applied to the study of PTCLs 3, 6, 14. Recent studies suggest that the T-cell phenotype is an independent significant prognostic factor, with PTCLs having one of the lowest overall survival and failure-free survival rates 3, 8, 10, 22.

Following publication of the REAL classification [1], we retrospectively analysed the clinical features of PTCL in 78 patients diagnosed according to the REAL classification to assess the clinical and pathological relevance.

Section snippets

Patient selection

Between October 1994 and March 1999, 78 patients with PTCLs were analysed. In all cases, Haematoxylin and Eosin (H&E) slides and immunostains for CD20 and CD3 (and CD30, CD56, etc., if needed), were performed and the diagnosis for subtype was made according to the REAL classification. For staging, bone marrow biopsy was immunostained with anti-CD3 and anti-CD20 antibodies. Mycosis fungoides, T-cell chronic lymphocytic leukaemia/prolymphocytic leukaemia, large granular lymphocytic leukaemia, and

Patient characteristics

The clinical characteristics of the 78 patients are given in Table 1. The median age was 44 years with a range of 15–77 years. The study involved 46 males (59%) and 32 females (41%). B symptoms were noted in 43 patients (55%).

The most common histopathological type was PTCL unspecified (PTCL-U), followed by angiocentric lymphoma, anaplastic large cell lymphoma (ALCL), angioimmunoblastic T-cell lymphoma (AILD), intestinal T-cell lymphoma, and subcutaneous panniculitic T-cell lymphoma, in

Discussion

The histological categorisation of lymphoma has been a source of frustration for many years. Using morphology, immunology, and genetic techniques, the REAL classification was proposed by the International Lymphoma Study Group [1]. However, the classification of PTCLs is still difficult and includes entities which are heterogenous in both biological nature and clinical features 3, 4, 6, 7, 11.

PTCLs have a striking epidemiological distribution with a lower incidence in Western countries than in

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