Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
Am J Cardiol. 2012 Nov 15;110(10):1507-11. doi: 10.1016/j.amjcard.2012.06.059. Epub 2012 Aug 3.
Cardiac involvement in systemic amyloidosis causes detrimental prognosis; therefore, early detection and classification are important to develop appropriate therapeutic strategies. Subcutaneous tissue biopsy is a useful screening procedure for systemic amyloidosis; however, its diagnostic and prognostic value in patients with cardiac amyloidosis remains elusive. Thus, we investigated the value of subcutaneous tissue biopsy in cardiac amyloidosis. In 22 patients with cardiac amyloidosis, we retrospectively analyzed 14 consecutive patients with cardiac amyloidosis who underwent subcutaneous tissue biopsy. Amyloid deposition was observed in 11 patients (79%). Histopathologic analysis demonstrated that acquired monoclonal immunoglobulin light-chain amyloidosis could be predicted when the degree of amyloid deposition was greater in blood vessels than adipose tissue compared to senile systemic amyloidosis and familial amyloidosis (60% vs 0%, p = 0.03). During the follow-up period (median 297 days, range 3 to 761), 7 patients (5 with monoclonal immunoglobulin light-chain amyloidosis and 2 with senile systemic amyloidosis) died or were admitted to the hospital because of worsening heart failure. Of them, 6 patients (86%) were positive for amyloid deposition in blood vessels in subcutaneous tissue biopsy. Incidence of death and composite outcome including heart failure hospitalization and death was significantly higher in patients positive for amyloid deposition in blood vessels than in those without (p = 0.03, p = 0.006, respectively). These results suggest that amyloid subtype could be diagnosed by assessing the degree of amyloid deposition in blood vessels and adipose tissue in subcutaneous tissue biopsy samples from patients with cardiac amyloidosis. Amyloid deposition in blood vessels suggests poor prognosis of these patients.
心脏淀粉样变可导致不良预后,因此早期发现和分类对于制定适当的治疗策略非常重要。皮下组织活检是一种用于系统性淀粉样变的有用筛查程序;然而,其在心脏淀粉样变患者中的诊断和预后价值仍不清楚。因此,我们研究了皮下组织活检在心脏淀粉样变中的价值。在 22 例心脏淀粉样变患者中,我们回顾性分析了 14 例连续接受皮下组织活检的心脏淀粉样变患者。在 11 例患者(79%)中观察到淀粉样物质沉积。组织病理学分析表明,当血管中淀粉样物质沉积程度大于脂肪组织时,与老年系统性淀粉样变性和家族性淀粉样变性相比,可以预测获得性单克隆免疫球蛋白轻链淀粉样变性(60%比 0%,p = 0.03)。在随访期间(中位数 297 天,范围 3 至 761 天),7 例(5 例单克隆免疫球蛋白轻链淀粉样变性和 2 例老年系统性淀粉样变性)因心力衰竭恶化或住院而死亡。其中,6 例(86%)患者的皮下组织活检中血管内有淀粉样物质沉积。血管内有淀粉样物质沉积的患者死亡和包括心力衰竭住院和死亡在内的复合终点的发生率明显高于无血管内淀粉样物质沉积的患者(p = 0.03,p = 0.006)。这些结果表明,通过评估心脏淀粉样变患者皮下组织活检样本中血管和脂肪组织内的淀粉样物质沉积程度,可以诊断淀粉样物质的亚型。血管内的淀粉样物质沉积提示这些患者的预后不良。