Figure 5.

Case 1. The clinical course in a patient with thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly (TAFRO) syndrome associated with adrenal hemorrhage and insufficiency. The clinical course in a 19-year-old Caucasian man included fever, platelet count, C-reactive protein (CRP), and creatinine levels. A partial response was obtained with tocilizumab injection. The patient was treated with high-dose steroids, followed by tocilizumab infusion. Due to persistent thrombocytopenia, second-line treatment commenced with rituximab, but the patient relapsed two months later. Tocilizumab treatment was recommenced, which was followed by an immuno-allergic adverse event. The patient then had a good response to sirolimus.