Kiguchi T, Sato C, Takai K, Nakai Y, Kaneko Y, Matsuki M
Department of Diagnostic Radiology, Ichinomiya Nishi Hospital, 1-Hira Kaimei, Ichinomiya City, Aichi 494-0001, Japan; Department of Diagnostic Radiology, Niigata City General Hospital, 463-7 Shumoku, Chuo-ku, Niigata City, Niigata 950-1197, Japan. Electronic address: takakig+
Department of Diagnostic Radiology, Niigata City General Hospital, 463-7 Shumoku, Chuo-ku, Niigata City, Niigata 950-1197, Japan.
Clin Radiol. 2017 Oct;72(10):905.e1-905.e5. doi: 10.1016/j.crad.2017.05.007. Epub 2017 Jun 16.
To assess detailed computed tomography (CT) findings in patients with the recently described thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly (TAFRO) syndrome, in order to contribute to imaging interpretation in the challenging diagnosis of this disease.
The institutional review board approved this retrospective study and waived the need for informed consent. Eleven patients (six men, five women; mean age, 52.5 years) with confirmed TAFRO syndrome were included in this study. Chest-to-pelvis CT images were analysed for the presence of anasarca, organomegaly, bone lesions, and lung lesions.
Anasarca was present in all patients and involved multiple cavities and tissues; pleural effusion and ascites were found in 100% of patients; pericardial effusion in 64%; periportal collar in 91%; gallbladder wall oedema in 78%; subcutaneous oedema in 91%; retroperitoneal oedema in 100%; and mesenteric oedema in 100%. Organomegaly involved multiple organs: hepatomegaly in 73%, splenomegaly in 82%, lymphadenopathy in 100%, and enlarged anterior mediastinum in 64% (solitary, well-circumscribed mass, 0%; infiltrative mass, 0%; non-mass-forming infiltrative lesion, 64%). Bone lesions were present in 91% patients and all bone lesions had ground-glass density with diffuse distribution. None of the patients had any lesions in their lungs.
The present study revealed that the findings of anasarca, organomegaly, and diffuse bony ground-glass appearance were observed in detail on CT in patients with TAFRO syndrome. A "matted" appearance of the enlarged anterior mediastinum is the characteristic CT finding of TAFRO syndrome, and it is possible to diagnose TAFRO syndrome from the combination of several CT findings.
评估近期描述的血小板减少、全身性水肿、发热、网状纤维增生和器官肿大（TAFRO）综合征患者的详细计算机断层扫描（CT）表现，以助力对该疾病进行具有挑战性的诊断时的影像解读。
机构审查委员会批准了这项回顾性研究，并免除了知情同意的要求。本研究纳入了11例确诊为TAFRO综合征的患者（6例男性，5例女性；平均年龄52.5岁）。分析胸部至骨盆的CT图像，以确定是否存在全身性水肿、器官肿大、骨病变和肺部病变。
所有患者均出现全身性水肿，累及多个腔隙和组织；100%的患者发现胸腔积液和腹水；64%有心包积液；91%有肝门部套袖征；78%有胆囊壁水肿；91%有皮下水肿；100%有腹膜后水肿；100%有肠系膜水肿。器官肿大累及多个器官：73%有肝肿大，82%有脾肿大，100%有淋巴结病，64%有前纵隔增宽（孤立性、边界清晰的肿块，0%；浸润性肿块，0%；非肿块形成的浸润性病变，64%）。91%的患者有骨病变，所有骨病变均为磨玻璃密度且分布弥漫。所有患者肺部均无病变。
本研究显示，TAFRO综合征患者的CT检查可详细观察到全身性水肿、器官肿大和弥漫性骨质磨玻璃样外观等表现。前纵隔增宽的“毡状”外观是TAFRO综合征的特征性CT表现，结合多项CT表现有可能诊断TAFRO综合征。
