血小板减少症,Anasarca,发烧,网织蛋白纤维化/肾衰竭,器官肿大包括TAFRO综合征,由于其积极的临床过程,它被认为是没有TAFRO综合征(iMCD-NOS)的iMCD的独特临床实体,对皮质类固醇的难治性,血小板减少症的存在,碱性磷酸酶水平升高,和正常水平的丙种球蛋白。然而,TAFRO综合征的早期诊断具有挑战性,因为它很罕见,诊断标准也很复杂.我们描述了一名患有TAFRO综合征和肾上腺出血的患者,她的临床病情迅速下降,对类固醇脉冲治疗没有反应。导致致命的结果。在她临床课程的早期阶段,她出现单侧肾上腺出血伴轻度血小板减少和凝血时间正常,提示肾上腺出血是TAFRO综合征的独特表现。总的来说,与iMCD-NOS患者相比,TAFRO综合征患者的临床病程更为积极,预后较差.为了改善这种不良预后,早期诊断疾病并立即开始使用强效免疫抑制剂如托珠单抗非常重要.基于这个案子,肾上腺出血可能提示TAFRO综合征,并促进这种复杂和罕见疾病的快速诊断。
Thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly comprise
TAFRO syndrome, which was proposed as a distinct clinical entity from iMCD without
TAFRO syndrome (iMCD-NOS) due to its aggressive clinical course, refractoriness to corticosteroids, presence of thrombocytopenia, increased level of alkaline phosphatase, and normal level of gammaglobulin. However, diagnosing TAFRO syndrome in its early stages is challenging because it is rare and its diagnostic criteria are complicated. We describe a patient with
TAFRO syndrome and adrenal hemorrhage who demonstrated a rapid decline in her clinical condition and did not respond to steroid pulse therapy, resulting in a fatal outcome. In the early stage of her clinical course, she developed unilateral adrenal hemorrhage with mild thrombocytopenia and normal clotting times, suggesting adrenal hemorrhage as a unique manifestation of TAFRO syndrome. In general, patients with
TAFRO syndrome exhibit a more aggressive clinical course and poorer outcome than those with iMCD-NOS. To ameliorate this poor prognosis, it is important to diagnose the disease early and immediately start powerful immunosuppressive agents such as tocilizumab. Based on this case, adrenal hemorrhage may suggest
TAFRO syndrome, and facilitate the rapid diagnosis of this complicated and rare disease.