野生型转甲状腺素蛋白相关弥漫性肺泡间隔淀粉样变性伴自发性肺血肿。
Diffuse Alveolar Septal Amyloidosis With Wild-Type Transthyretin With Spontaneous Lung Hematoma.
机构信息
Department of General Thoracic Surgery, Takarazuka City Hospital, Takarazuka, Japan.
Department of Pathology and Laboratory Medicine, Takarazuka City Hospital, Takarazuka, Japan.
出版信息
Ann Thorac Surg. 2019 Feb;107(2):e131-e133. doi: 10.1016/j.athoracsur.2018.05.096.
We experienced a mass formation in the right lower lobe in a patient with cardiac amyloidosis and heart failure. Radiologic findings of the chest showed no abnormality except a mass. The patient had non-valvular atrial fibrillation and was taking edoxaban. Surgical resection of the mass revealed a hematoma. Further pathologic evaluation revealed diffuse alveolar septal amyloidosis with transthyretin (ATTR). The genetic testing found no mutation in the TTR gene. Therefore, systemic wild-type TTR amyloidosis (ATTRwt) was confirmed. Alveolar septal ATTRwt is rare and patient had alveolar septal ATTRwt with spontaneous lung hematoma.
我们在一位患有心脏淀粉样变性和心力衰竭的患者的右下肺叶中观察到了一次肿块形成。胸部的放射学发现除了肿块外没有异常。患者患有非瓣膜性心房颤动，正在服用依度沙班。肿块的手术切除显示为血肿。进一步的病理评估显示弥漫性肺泡间隔淀粉样变性伴转甲状腺素（ATTR）。基因检测未发现 TTR 基因的突变。因此，确诊为全身性野生型 TTR 淀粉样变性（ATTRwt）。肺泡间隔 ATTRwt 较为罕见，且该患者患有肺泡间隔 ATTRwt 并伴有自发性肺血肿。
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