A 58-year-old woman was admitted to our hospital with anasarca and generalized lymphadenopathy. Laboratory data showed serum creatinine 1.48 mg/dL, C-Reactive Protein 2.38 mg/dL, platelet 102,000/μL, and anti-SS-A antibodies. A lymph node biopsy revealed a regressed germinal center and hypervascularization in the interfollicular area. The patient was diagnosed with TAFRO-like symptoms occurring with Sjögren's syndrome and human T-cell leukemia virus type 1 (HTLV-1) infection, and 80 mg of methylprednisolone and tocilizumab 8 mg/kg biweekly were initiated. Her body weight decreased from 59.4 to 41 kg, and pleural effusion disappeared 8 weeks later. This case suggests that TAFRO-like symptoms may occur in patients with Sjögren's syndrome with HTLV-1 infection.
Keywords: HTLV-1; Sjögren's syndrome; TAFRO syndrome.