ALK‐negative anaplastic large‐cell lymphoma demonstrates similar poor prognosis to peripheral T‐cell lymphoma, unspecified

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Abstract
Aims: Anaplastic large cell lymphoma (ALCL) is classically considered a clinicopathological entity separate from other nodal mature T‐cell lymphomas (TCL). Recently, the anaplastic lymphoma kinase (ALK) protein was shown to identify a subgroup of nodal ALCL with an excellent prognosis, whereas ALK‐negative ALCLs are more heterogeneous. The aim of this study was to investigate the clinicopathological parameters in relation to clinical behaviour of ALK‐negative ALCL compared with other nodal mature TCL, i.e. peripheral TCL, unspecified (PTCL‐NOS) and angioimmunoblastic lymphoma (AILT). Methods and results: Clinicopathological data of ALK‐positive (n = 28) and ALK‐negative (n = 46) ALCL; PTCL‐NOS (n = 47); and AILT (n = 12) were analysed for their prognostic significance. While ALK‐positive ALCL shows favourable clinical features and a good prognosis, ALK‐negative ALCL, PTCL‐NOS and AILT are all associated with high age groups, advanced disease stage, and poor prognosis (<45% 5‐year survival). In multivariate analysis of overall survival time, performed in the combined group of ALK‐negative nodal mature T‐cell lymphomas, only age and the International Prognostic Index (IPI) remained independent prognostic parameters, while lymphoma subtype (ALCL versus PTCL‐NOS versus AILT) gave no additional information. Conclusions: The distinction between ALK‐negative ALCL and PTCL‐NOS or AILT is of limited clinical relevance as they show comparable poor prognosis. In these lymphoma subtypes, only age and the IPI are of significant prognostic value.

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