心脏淀粉样变患者心内膜心肌活检的临床分析  

Clinical summary of endomyocardial biopsy in patients with cardiac amyloidosis

作　　者：武丽娜[1] 朱虹 潘小宏[2] Wu Lina;Zhu Hong;Pan Xiaohong(Department of Cardiology,the Fifth Affiliated Hospital,Zhengzhou University,Zhengzhou 450052,China;不详)

机构地区：[1]郑州大学第五附属医院心血管内科,郑州450052 [2]浙江大学医学院附属第二医院心血管内科,杭州310000

出　　处：《中国循证心血管医学杂志》2024年第11期1303-1305,共3页Chinese Journal of Evidence-Based Cardiovascular Medicine

基　　金：河南省医学科技攻关计划联合共建项目(LHGJ20220556)。

摘　　要：目的探讨某三甲医院心脏淀粉样变性患者心内膜心肌活检的临床应用和经验。方法回顾性分析42例经心内膜心肌活检确诊心脏淀粉样变性患者临床表现及心内膜心肌活检病理结果特点。结果心内膜心肌活检病理结果主要表现为光镜下刚果红染色阳性,偏振光可见苹果绿双折射,电镜下间质可见淀粉样纤维沉积,经免疫组化、免疫电镜,部分患者行质谱分析、基因检测,最终明确诊断免疫球蛋白轻链型40例,其中λ型34例,κ型6例,转甲状腺素蛋白型2例。免疫球蛋白轻链型患者给予化疗方案包括硼替佐米、地塞米松、环磷酰胺。转甲状腺素蛋白型患者口服氯苯唑酸治疗。结论临床怀疑心脏淀粉样变性时应尽早行心内膜心肌活检,明确诊断及分型,以指导临床治疗。Objective To investigate the clinical application and experience of endomyocardial biopsy in patients with cardiac amyloidosis in a third class hospital.Methods The clinical manifestations and pathological findings of 42 patients with cardiac amyloidosis confirmed by endomyocardial biopsy were retrospectively analyzed.Results The pathological results of endomyocardial biopsy were mainly positive for Congo red staining under light microscope,apple green birefringence under polarized light,and amyloid fiber deposition in the stroma under electron microscopy.By immunohistochemistry,immunoelectron microscopy,mass spectrometry and gene detection were performed in some patients,40 cases of immunoglobulin light chain type were identified,including 34 cases ofλtype,6 cases ofκtype,and 2 cases of transthyretin type.Patients with light-chain immunoglobulin were given chemotherapy regimens,including bortezomib,dexamethasone,and cyclophosphamide.Patients with transthyretin protein type were treated with tafamidis.Conclusion When clinically suspected cardiac amyloidosis,endomyocardial biopsy should be performed as soon as possible to confirm diagnosis and classification and guide treatment.

关 键 词：心内膜心肌活检 心脏淀粉样变性 临床应用 

分 类 号：R542.4[医药卫生—心血管疾病]