Pathobiology of Mature T-Cell Lymphomas

Mature T- and natural killer (NK)—cell neoplasms are relatively rare forms of leukemia/lymphoma. The diagnosis of these entities is often difficult, necessitating extensive immunophenotypic, molecular, and genetic testing. Despite the accumulating information on the pathobiology of these neoplasms, in many cases the prognosis remains poor. This article presents an updated view of the morphologic, immunophenotypic, genetic, and molecular characteristics of the mature T- and NK-cell neoplasms. For a better understanding of this complex topic, the development of normal T and NK cells is briefly discussed. The presentation of the characteristic features of the neoplasms in the 2008 World Health Organization classification of hematopoietic neoplasms includes advances in the understanding of the pathobiology of each diagnostic category.