Diagnostic value of abdominal wall fat pad biopsy in senile systemic amyloidosis

Diagnostic value of abdominal wall fat pad biopsy in senile systemic amyloidosis
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DOI:
10.3109/13506129.2011.623199
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发表时间:
2011-12-01
影响因子:
5.5
通讯作者:
Koyama, Jun
Koyama, Jun
中科院分区:
医学2区
文献类型:
--
作者:
Ikeda, Shu-ichi;Sekijima, Yoshiki;Koyama, Jun

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老年性系统性淀粉样变性(SSA)是老年人顽固性心力衰竭的主要原因。为了证实经甲状腺素(TTR)衍生的淀粉样蛋白沉积,通常在SSA患者中进行心内膜活检,但这种侵入性活检技术并不总是适用于严重心功能障碍的老年患者。在过去的3年中,我们检查了11例SSA患者(6男5女,年龄从70岁到97岁)。所有患者均行腹壁皮肤活检,8例显示trr免疫反应性淀粉样蛋白沉积(敏感性:73%):淀粉样蛋白沉积主要见于皮下脂肪组织深层,呈斑片状分布。它们是弱亲和性的,但被抗ttr抗体强烈免疫标记。SSA患者活检中淀粉样蛋白沉积的严重程度和模式与年龄匹配的ttr相关家族性淀粉样蛋白多发性神经病患者有很大不同。外科皮肤活检包括深皮下脂肪垫可以安全地在床边进行,对SSA的组织病理学诊断很有用。
Senile systemic amyloidosis (SSA) is a main cause of intractable heart failure in elderly individuals. To demonstrate transthyretin (TTR)-derived amyloid deposition endomyocardial biopsy has been commonly carried out in the patients with SSA, but this invasive biopsy technique cannot always be performed in aged patients with severe cardiac dysfunction. During the past 3 years, 11 patients with SSA (6 males and 5 females; ages from 70 to 97 years) were examined. All underwent skin biopsy from the abdominal wall and 8 showed TTR-immunoreactive amyloid deposition (sensitivity: 73%): amyloid deposits were seen mainly in the deep layer of subcutaneous fat tissue and showed a patchy distribution. They were weakly Congophilic, but were strongly immunolabeled by an anti-TTR antibody. The severity and pattern of amyloid deposition in this biopsy of SSA patients were considerably different from those obtained from age-matched patients with TTR-related familial amyloid polyneuropathy. Surgical skin biopsy including the deep subcutaneous fat pad can be performed safely at the bedside and is useful for the histopathological diagnosis of SSA.