Department of Hematology and Oncology, Okayama University Hospital, Japan.
Department of Clinical and Laboratory Medicine, University of Yamanashi, Japan.
Intern Med. 2024;63(13):1917-1922. doi: 10.2169/internalmedicine.2799-23. Epub 2024 Jul 1.
Thrombocytopenia, anasarca, fever, renal dysfunction, and organomegaly (TAFRO) syndrome is an inflammatory disorder with an unclear pathogenesis. We herein report a case of TAFRO syndrome in remission in a patient who experienced recurrent intracranial bleeding despite a normal platelet count and coagulation system. A further investigation suggested the presence of anti-glycoprotein VI (GPVI) autoantibodies in the plasma, which induced platelet dysfunction and bleeding tendency. No new bleeding or relapse of TAFRO syndrome occurred after immunosuppressive therapy was initiated. These findings may help elucidate the autoimmune pathogenesis of TAFRO syndrome.
血小板减少症、浮肿、发热、肾功能障碍和器官肿大(TAFRO)综合征是一种发病机制尚不明确的炎症性疾病。本文报告一例 TAFRO 综合征缓解病例,该患者尽管血小板计数和凝血系统正常,但仍反复发生颅内出血。进一步研究提示血浆中存在抗糖蛋白 VI(GPVI)自身抗体,导致血小板功能障碍和出血倾向。免疫抑制治疗后未再发生新的出血或 TAFRO 综合征复发。这些发现可能有助于阐明 TAFRO 综合征的自身免疫发病机制。