Difficult-to-treat TAFRO syndrome associated with Sjögren’s syndrome successfully treated with Dual B cell depletion therapy: A case report

Abstract TAFRO syndrome is a systemic inflammatory disease of unknown etiology. It is characterized by thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly. Herein, we report the case of a 60-year-old male with TAFRO syndrome. A few weeks after the patient developed an intermittent fever, he presented to our hospital with diarrhea, abdominal distension, and whole-body edema (face, extremities, and abdomen). Autoantibody and lip biopsy findings supported the diagnosis of primary Sjögren’s syndrome (pSS). High-dose steroids and tocilizumab were used to treat his refractory thrombocytopenia and ascites. However, systemic inflammation and renal dysfunction did not improve, resulting in temporary hemodialysis. Eventually, dual B-cell depletion therapy with rituximab and belimumab ameliorated the patient’s symptoms. About 16 weeks after discharge, the patient’s overall condition had improved. The TAFRO syndrome may be a severe manifestation of pSS. Considering the immunological context, B cell-targeted therapy provides new insights into improving this life-threatening disease and enables rapid steroid tapering.