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