Angioimmunoblastic T-cell lymphoma is the most common T-cell lymphoma in two distinct French information data sets

Abstract

Reliable information regarding the current prevalence of peripheral T-cell lymphoma (PTCL) entities is missing. Herein we report on the frequency of PTCL entities in France between 2010 and 2013. Using Lymphopath, a national lymphoma network established by the French National Cancer Agency, which covers about 70 % of all lymphomas currently diagnosed in France, we found that PTCL comprised 6.5 % of non-cutaneous lymphomas with angioimmunoblastic T-cell lymphoma (AITL) being the most frequent (739 cases; 36 %), followed by peripheral Tcell lymphoma not otherwise specified (PTCL-NOS) (550 cases, 27%). These data were verified in an independent data set from a transnational research consortium active in three European countries. In comparison to epidemiologic data reported previously, we show that AITL is by far the most common PTCL subtype. In light of the results of recent molecular findings highlighting the heterogeneity of T-cell lymphomas and the advent of targeted therapies, these data have important implications for both basic and clinical research.Peripheral T-cell lymphomas (PTCLs) represent diverse and complex diseases, estimated to represent an overall 10-15% of all lymphomas worldwide, with the highest incidence rates occurring in Asia.1,2 The relative prevalence of PTCL entities delineated according to the criteria of the REAL (1994) /WHO (2001) classification systems, was evaluated in multiple institutions in the late 1990s and early 2000s, based on retrospective cohorts of patients. In those series, PTCL-NOS (a "by default" diagnosis for cases not fulfilling the criteria for other more specific entities) was the most frequent entity, followed by anaplastic large cell lymphoma (ALCL) and AITL, while extranodal entities, in general, accounted for a small proportion of the cases. 1,3,4 This worldwide epidemiology was most recently addressed by the International PTCL study, which reviewed more than 1,300 patients diagnosed with PTCL between 1990 and 2002 in North America, Europe and Asia.5 In this cohort, PTCL-NOS was the most common diagnosis (25,9%), followed by AITL (18,5%), representing 30.4% and 21.7% of non-cutaneous PTCL, respectively (Figure 1). 5This study also confirmed geographic variations in the distribution of PTCL entities, notably demonstrating that the highest frequencies of AITL and enteropathy-associated Tcell lymphoma (EATL) are in Europe.Here, we provide recent data obtained from a large prospective survey in France. The prevalent analysis of PTCL was derived from data collected through Lymphopath, a national network of 33 expert reference centers for hematopathology which was established by the French National Cancer Agency in 2010. Non-expert pathologists are encouraged to refer every newly diagnosed lymphoma for review to a Lymphopath center. Diagnoses provided by experts, following slide review and additional ancillary techniques performed in the reference center, are entered into a central database. Of the 31,401 non-cutaneous lymphomas reviewed in Lymphopath over four years (20...

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