A superelderly case of TAFRO syndrome treated effectively using corticosteroid hormones

Castleman disease (CD) is a rare systemic disorder classified under atypical lymphoproliferative disorders. Multicentric CD (MCD) is its subtype wherein patients develop multiple lesions and systemic symptoms 1. Thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome is a new disease concept, which has been named based on the thrombocytopenia, anasarca, myelofibrosis, renal failure, and organomegaly 2, 3. Architectural features typical of unicentric hyaline vascular CD are not observed in patients with TAFRO syndrome; furthermore, enlarged nuclei are found in proliferating endothelial cells at germinal centers and the interfollicular zone of such patients 2. The median age of disease onset is 50 years (range, 23–72) 4. Complete remission rates following corticosteroid therapy with/without interleukin (IL)‐6‐targeting strategies are lower in TAFRO syndrome than those in classical MCD 2. Here, we report a superelderly patient with TAFRO syndrome who developed high‐grade fever and an acute disease that could have been fatal within weeks. Her clinical symptoms markedly improved on treatment with prednisolone (PSL) alone.