Fatal TAFRO syndrome in which adrenal hemorrhage preceded thrombocytopenia

TAFRO syndrome is a rare systemic inflammatory disease characterized by thrombocytopenia, anasarca, fever, renal dysfunction, and myelofibrosis, and its follows a rapidly fatal clinical course. A few cases of TAFRO syndrome complicated by adrenal hemorrhage were recently reported; however, its pathogenetic significance remains largely unclear.The present case was a 59-year-old man presenting with left lower back pain. Imaging studies revealed left adrenal hemorrhage. Although an elevated C-reactive protein level was observed in the initial visit, there were no obvious abnormalities in the platelet count, renal function, or coagulation profile. Subsequently, fluid retention and renal dysfunction progressed rapidly, and thrombocytopenia emerged, leading to a diagnosis of TAFRO syndrome. Treatment including glucocorticoid therapy was initiated, but was ineffective; the disease progressed rapidly, and the patient succumbed to multiple organ failure. Autopsy revealed findings consistent with TAFRO syndrome in small lymph nodes and those suggestive of thrombotic microangiopathy in renal glomeruli.Based on these findings, adrenal hemorrhage may occur as an initial presentation prior to the manifestation of the full systemic picture of TAFRO syndrome. Furthermore, the disease progressed rapidly to a severe state in a short period without initial thrombocytopenia-which complicated the diagnosis-and demonstrated resistance to conventional treatments including glucocorticoids, which underscores the importance of early recognition and therapeutic intervention.