Department of Hematology/Oncology, Wakayama Medical University, Wakayama 6418509, Japan.
Department of Internal Medicine, Naga Municipal Hospital, Wakayama 6496414, Japan.
Medicina (Kaunas). 2021 Sep 16;57(9):971. doi: 10.3390/medicina57090971.
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 × 10/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.
TAFRO 综合征是特发性多中心 Castleman 病的一种罕见亚型，表现为血小板减少、全身浮肿、发热、网状纤维纤维化和器官肿大。血栓性微血管病，包括肾功能障碍，常与该综合征相关。TAFRO 综合征可能危及生命并迅速进展，其诊断和管理仍然具有挑战性。一名 48 岁女性因临床和组织病理学发现被诊断为并发血栓性微血管病的 TAFRO 综合征。在接受大剂量类固醇治疗后，她的血小板减少和全身浮肿并未改善。随后，患者接受环孢素 A 和利妥昔单抗联合二线治疗，临床症状显著逐渐改善。同时，她的血小板计数增加到超过 40×10/L；然而，她出现了颅内出血。行手术清除血肿后，患者恢复并达到持续缓解。根据这些发现，即使对 TAFRO 综合征进行强化治疗，也应注意与局部血栓性微血管病相关的危及生命的出血。
