Treating life-threatening TAFRO syndrome with interleukin-1 inhibition

Abstract

TAFRO syndrome (Thrombocytopenia, Anasarca, Fever, Reticulin fibrosis, and Organomegaly) is a newly recognized variant of idiopathic - human herpesvirus-8 (HHV-8) negative - Multicentric Castleman Disease (iMCD) [1]. Although salient clinical and histopathological features are similar to most of iMCD related disorders, TAFRO syndrome follows a more aggressive course characterized by rapidly progressive multiorgan failure [2]. Due to its recent recognition, standard treatments for TAFRO syndrome are lacking and mostly derived from immunosuppressive regimens used in iMCD including corticosteroids, chemotherapeutic agents (thalidomide and bortezomib), and monoclonal antibodies targeting CD20+ B-lymphocytes (rituximab) or interleukin (IL)-6 (siltuximab and tocilizumab) [1].