Ruxolitinib in relapsed/refractory TAFRO syndrome: a report of two cases, including one positive for Epstein-Barr virus, and a literature review.

TAFRO syndrome is a rare systemic inflammatory disorder characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly. Its pathogenesis remains elusive, and its non-specific symptoms overlap with idiopathic multicentric Castleman disease, complicating its management. This report discusses two cases of TAFRO syndrome resistant to conventional treatments, including corticosteroids, tocilizumab, and rituximab. Both patients showed significant clinical improvement after treatment with ruxolitinib, a JAK-STAT pathway inhibitor. The first patient was a 55-year-old man who showed clinical improvement with ruxolitinib after the failure of multiple lines of treatment, including corticosteroids, tocilizumab, rituximab, and cyclosporin A. The second was another 55-year-old man who experienced disease relapse 18 months after initial treatment but responded rapidly to ruxolitinib. These cases highlight the potential role of ruxolitinib as a therapeutic option for refractory TAFRO syndrome. In addition, Epstein-Barr virus (EBV) DNA was detected in the serum of the first patient, making this the first report to demonstrate the effectiveness of ruxolitinib in EBV-associated TAFRO syndrome. These findings highlight the need for further studies to confirm the efficacy of ruxolitinib in such cases and elucidate the pathophysiological mechanisms underlying TAFRO syndrome, which could guide future therapeutic strategies.