TAFRO is an acrostic and includes thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R) and organomegaly (O) (Takai et al., 2013). TAFRO syndrome has been described firstly by Takai in Japanese patients. However TAFRO cases have been reported from US, Europe and other countries (Takai et al., 2010; Iwaki et al., 2016; Abdo et al., 2014). Three major and at least one minor criteria and exclusion of infectious, rheumatologic and neoplastic diseases are required for the diagnosis of TAFRO. In fact TAFRO must be thought in clinically undiagnosed and unsolved problemmatic cases.