TAFRO syndrome with a rapid fatal course despite corticosteroid and tocilizumab therapy

Abstract

TAFRO syndrome is a recently proposed subtype of multicentric Castleman’s disease. Some patients have a refractory and aggressively fatal course. Here we report a case of TAFRO syndrome with a rapid fatal course despite corticosteroid and tocilizumab therapy. A 68-year-old Japanese male suffered from abdominal pain, watery diarrhea, fever, and general fatigue. On the 12th hospital day, severe thrombocytopenia, anasarca, and renal failure developed. The histopathological findings of lymph node biopsy were indicative of MCD. Although corticosteroid and tocilizumab were administered, the patient died of multiple organ failure on the 30th hospital day. Autopsy findings revealed that inflammation due to TAFRO syndrome was suppressed but multiple infarctions progressed and became the direct cause of death. Our case suggested that TAFRO syndrome should not be excluded even if clinical findings are insufficient for the diagnostic criteria at the onset and that in patients with TAFRO syndrome who are at a high risk of thrombosis, multiple infarctions may become a direct cause of death.