The spectrum of lymphoblastic, nodal and extranodal T-cell lymphomas: characteristic features and diagnostic dilemmas

Abstract

T-cell lymphomas represent a heterogeneous group of neoplasms that encompass considerable clinical, morphologic, and immunophenotypic variation. The diagnosis of T-cell lymphoma is challenging because of its relative rarity, the lack of an immunophenotypic marker of clonality, and significant morphologic overlap with infectious/inflammatory processes and neoplasms, including Hodgkin and other non-Hodgkin lymphomas, and even mesenchymal or epithelial lesions. In the current World Health Organization classification of hematopoietic tumors, all except 1 subtype (ie, T-lymphoblastic lymphoma) are recognized as mature neoplasms derived from postthymic T cells. In addition to T-lymphoblastic lymphoma, this review will focus on nodal and extranodal T-cell lymphomas and exclude T-cell lymphomas presenting primarily in the skin. Extranodal natural-killer-cell/T-cell lymphoma, nasal type, will also be discussed because the derivation of this lymphoma from natural killer and natural killer-like T cells shows morphologic and immunophenotypic features that overlap with other T-cell lymphomas. In this review, we discuss the salient clinicopathologic, immunophenotypic, and genetic features, as well as our approaches to the diagnosis of lymphoblastic, nodal, and extranodal T-cell lymphomas.