Successful Treatment of TAFRO (Thrombocytopenia, Anasarca, Fever, Renal Insufficiency, and Organomegaly) Syndrome With Triple Combination Therapy of Corticosteroid, Tocilizumab, and Cyclosporine: A Case Report

We report a case of the most severe grade TAFRO (thrombocytopenia, anasarca, fever, renal insufficiency, and organomegaly) syndrome that was successfully treated with triple combination therapy of corticosteroid, tocilizumab and cyclosporine. A 48-year-old man with a history of thrombotic thrombocytopenic purpura was referred to our department for dyspnea, pleural effusion, and ascites. Further examination revealed thrombocytopenia (platelet count 4.7×10⁴/μL), severe renal dysfunction (creatinine 2.56 mg/dL), and systemic inflammation (C-reactive protein 18.52 mg/dL). Computed tomography revealed mild lymphadenopathy and mild hepatomegaly. Bone marrow examination showed hypocellularity and increased reticulin fibrosis, and lymph node biopsy showed proliferation of endothelial venules. Thus, the patient was diagnosed with TAFRO syndrome. After the combination of steroid pulse therapy (intravenous methylprednisolone 1 g/day for three days and subsequent oral prednisolone 60 mg/day), tocilizumab at 480 mg once weekly, and cyclosporine at 225 mg once daily, systemic inflammation and kidney function gradually improved. This case suggests that the triple combination therapy can lead to remission of the most severe grade TAFRO syndrome.