Intracranial Hemorrhage in a Patient with TAFRO Syndrome Treated with Cyclosporine A and Rituximab

Abstract

TAFRO syndrome, a rare subtype of idiopathic multicentric Castleman disease, manifests as thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. Thrombotic microangiopathy, including renal dysfunction, is frequently associated with this syndrome. TAFRO syndrome can be life threatening and show rapid progression, and the diagnosis and management of this disorder remain challenging. A 48-year-old woman was diagnosed with TAFRO syndrome complicated by thrombotic microangiopathy based on the clinical and histopathological findings. After receiving high-dose steroids, her thrombocytopenia and anasarca did not improve. The patient subsequently received a combination of cyclosporine A and rituximab as second-line therapy, which resulted in a significant gradual improvement in the clinical symptoms. Meanwhile, her platelet count increased to more than 40 × 109/L; however, she developed intracranial hemorrhage. Following surgical evacuation, the patient recovered with an achievement of sustained remission. Based on these findings, attention should be paid to life-threatening bleeding associated with local thrombotic microangiopathy even when intensive treatment is administered for TAFRO syndrome.