[Current standard in diagnostic and therapy of peripheral T-cell lymphoma]

NEW WHO CLASSIFICATION 2016 FOR LYMPHOMA AND MOLECULAR MARKER:  The new WHO 2016 Classification for Lymphomas included more detailed definitions of individual entities, such as: the Anaplastic Large Cell Lymphoma, ALK; the terminus of enteropathy-associated T-cell lymphoma (EATCL, type 1) now is restricted to celiac-associated form. The integration of next Generation sequencing (NGS) enables better differential diagnostics, e. g. angioimmunoblastic lymphoma (AITL) can be characterized by the presence of TET2, RHOA, IDH2 or DNMT3A mutations. Furthermore, certain mutations also have prognostic impact. In ALCL/ALK-, evidence of a DUSP22 / IRF4 re-arrangement is associated with a better and detection of TP63 is associated with a worse prognosis.

In addition to B symptoms and nodal manifestation, extranodal manifestations such as skin infiltrates can be observed in up to 20 %. In AITL, polyclonal hypergammaglobulinemia, Coombs-positive hemolytic anemia and immunodeficiency can occur, as a sign of a dysregulated immune system.

By combining the immunoconjugate brentuximab-vedotin with chemotherapy, improvement in disease-free and overall survival in CD30⁺-PTCL (especially in ALCL) was observed.

At present demethylating agent 5-azacytidine is explored in AITL and PTCL with a T-follicle helper type (TFH).