Abstract
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).
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