Effective treatment with ruxolitinib and ropeginterferon alfa-2b for refractory TAFRO- like syndrome in Polycythemia vera

TAFRO syndrome is a rare systemic inflammatory disease characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis, and splenomegaly; however, its pathogenesis remains largely unknown. Due to the lack of appropriate treatment, TAFRO syndrome often presents with multiple organ dysfunction and fatality. The Janus kinase (JAK)/signal transducers and activators of transcription (STAT) pathway has recently been shown to play an important role in the pathogenesis of inflammation in idiopathic Multicentric Castleman disease (iMCD)-TAFRO, and inhibitors of the JAK/STAT pathway may be effective as therapeutic agents for iMCD-TAFRO syndrome. Polycythemia vera (PV) is one of the Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs), and the enhancement and modulation of immune cells may be involved in the mechanism of action of interferon (IFN)-α2 and the JAK-STAT pathway in MPNs. We herein report the successful treatment using combination therapy with ruxolitinib and ropeginterferon alfa-2b of a case of TAFRO-like syndrome with a long history of PV with JAK2 V617F refractory to several treatments. This combination therapy has potential as a new treatment option for refractory TAFRO syndrome-like symptoms.