Kurokawa Ryo, Baba Akira, Kano Rui, Kaneko Yo, Kurokawa Mariko, Gonoi Wataru, Abe Osamu
Department of Radiology, Graduate School of Medicine, The University of Tokyo, 7-3-1, Hongo, Bunkyo-ku, Tokyo 113-8655, Japan.
Department of Radiology, The Jikei University School of Medicine, 3-25-8, Nishi-Shimbashi, Minato-ku, Tokyo 105-8461, Japan.
Biomedicines. 2024 Apr 10;12(4):837. doi: 10.3390/biomedicines12040837.
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.
本系统评价文章旨在研究血小板减少、全身性水肿、发热、网状纤维增生、肾功能不全及器官肿大（TAFRO）综合征患者肾上腺异常的临床及放射影像学特征。我们检索了PubMed、Cochrane图书馆及Web of Science核心合集数据库中的文献。最终，我们分析了11项研究，共22例患者，加上我们的1例患者，总计23例患者。平均年龄为47.0±12.6岁。男性患者20例，女性患者3例。15例患者（65.2%）进行了淋巴结组织病理学分析，所有15例患者的诊断均符合TAFRO综合征。在这23例患者中，11例患者（18个肾上腺）表现为肾上腺缺血/梗死，9例患者（13个肾上腺）表现为肾上腺出血，4例患者（7个肾上腺）表现为肾上腺肿大，且无并发缺血/梗死或出血的证据。1例患者表现为单侧肾上腺出血及对侧肾上腺肿大。肾上腺缺血/梗死患者的肾上腺在对比增强计算机断层扫描（CT）中强化不佳。肾上腺出血患者的肾上腺在非增强CT中表现为高密度影，在磁共振成像中表现为血肿。所有患者（23/23，100%）均观察到肾上腺肿大，无论是否伴有肾上腺缺血/梗死或出血。对受累肾上腺进行随访CT检查时，常可见随后的钙化（9/14，64.3%）。腹痛较为常见（15/23，65.2%），所有腹痛均在疾病发作后出现，提示将TAFRO综合征视为急腹症病因的重要性。鉴于非TAFRO型特发性多中心Castleman病（iMCD）中无肾上腺异常的证据，肾上腺异常可作为区分TAFRO综合征与非TAFRO型iMCD的诊断线索。
