PDF(Pubmed)
Abstract：
This systematic review article aims to investigate the clinical and radiological imaging characteristics of adrenal abnormalities in patients with thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome. We searched the literature in PubMed, the Cochrane Library, and the Web of Science Core Collection. Ultimately, we analyzed 11 studies with 22 patients plus our 1 patient, totaling 23 patients. The mean age was 47.0 ± 12.6 years. There were 20 male and 3 female patients, respectively. The histopathological analysis of lymph nodes was conducted in 15 patients (65.2%), and the diagnosis was consistent with TAFRO syndrome in all 15 patients. Among the 23 patients, 11 patients (18 adrenal glands) showed adrenal ischemia/infarction, 9 patients (13 adrenal glands) showed adrenal hemorrhage, and 4 patients (7 adrenal glands) showed adrenomegaly without evidence of concurrent ischemia/infarction or hemorrhage. One patient demonstrated unilateral adrenal hemorrhage and contralateral adrenomegaly. In patients with adrenal ischemia/infarction, the adrenal glands displayed poor enhancement through contrast-enhanced computed tomography (CT). In patients with adrenal hemorrhage, the adrenal glands revealed high attenuation through non-enhanced CT and hematoma through magnetic resonance imaging. Adrenomegaly, with or without adrenal ischemia/infarction or hemorrhage, was observed in all patients (23/23, 100%). The subsequent calcification of the affected adrenal glands was frequently observed (9/14, 64.3%) when a follow-up CT was performed. Abdominal pain was frequent (15/23, 65.2%), all of which occurred after the disease\'s onset, suggesting the importance of considering TAFRO syndrome as a cause of acute abdomen. Given the absence of evidence of adrenal abnormalities in non-TAFRO-idiopathic multicentric Castleman disease (iMCD), they may serve as diagnostic clues for differentiating TAFRO syndrome from non-TAFRO-iMCD.
摘要：
本文旨在探讨血小板减少症患者肾上腺异常的临床及影像学特点。Anasarca,发烧,网状蛋白纤维化，肾功能不全,和器官肿大（TAFRO）综合征。我们搜索了PubMed的文献，Cochrane图书馆,和WebofScience核心收藏。最终,我们分析了11项研究，包括22名患者和我们的1名患者，共23名患者。平均年龄为47.0±12.6岁。有20名男性和3名女性患者，分别。15例(65.2%)患者进行淋巴结组织病理学分析,所有15例患者的诊断与TAFRO综合征一致。在23名患者中，11例患者（18个肾上腺）表现为肾上腺缺血/梗塞，9例（13例肾上腺）显示肾上腺出血，4例患者（7个肾上腺）表现出肾上腺肿大，没有并发缺血/梗塞或出血的证据。一名患者表现为单侧肾上腺出血和对侧肾上腺肿大。在肾上腺缺血/梗塞患者中，通过对比增强计算机断层扫描（CT），肾上腺显示增强不良。在肾上腺出血患者中，肾上腺通过非增强CT显示高度衰减，通过磁共振成像显示血肿。肾上腺肿大,有或没有肾上腺缺血/梗塞或出血，在所有患者中观察到（23/23，100%）。进行CT随访时，经常观察到受影响的肾上腺的随后钙化（9/14，64.3％）。腹痛频发（15/23，65.2%），所有这些都发生在疾病发作后，提示将TAFRO综合征视为急腹症病因的重要性。鉴于在非TAFRO特发性多中心Castleman病（iMCD）中没有肾上腺异常的证据，它们可以作为鉴别TAFRO综合征和非TAFRO-iMCD的诊断线索.
