关键词: 淀粉样变性, 心肌, 肌钙蛋白, 超声心动描记术, 压力, 活组织检查

Abstract: Background　The immunoglobulin amyloid light-chain cardiac amyloidosis （AL-CA） has a rapid progress and a high mortality rate，but its clinical characteristics are not fully recognized at present.Objective　To summarize the clinical data of immunoglobulin AL-CA patients to improve clinicians' awareness of this disease.Methods　Forty-three inpatients with AL-CA from Beijing Chao-yang Hospital，Capital Medical University were enrolled from January 2012 to December 2016.A retrospective analysis was performed on their general data，clinical symptoms and signs，laboratory examination，12-lead electrocardiogram，echocardiogram，cardiac magnetic resonance imaging，biopsy and therapeutic strategies.Results　（1）The mean onset age was （59±10） years old.Men had a higher prevalence rate〔74.4%（32/43）〕．Type λ was the most prevalent〔69.8% （30/43）〕．25 cases （58.1%） were accompanied with multiple myeloma.（2） Symptoms observed during admission were various.Clinical manifestations mainly consisted of chest tightness and shortness of breath 〔18 cases（41.9%）〕，edema of lower extremity 〔9 cases（20.9%）〕 and tiredness 〔9 cases（20.9%）〕.（3） The increase in BNP〔3 474（1 079，7 040）ng/L〕and cTnI〔0.21（0.06，1.47）μg/L〕 were common in laboratory tests，some of which were accompanied with decreased hemoglobin 〔13 cases（30.2%） 〕and elevated serum creatinine〔32 cases（74.4%）〕.The most common manifestations of electrocardiogram were pathologic Q wave 〔12 cases（27.9%）〕，low voltage of the limb leads 〔11 cases（25.6%）〕 and poor R wave progression in precordial leads 〔6 cases（14.0%）〕.Dysrhythmia and conduction block were also be found in some cases.Echocardiography showed atrial enlargement 〔34 cases（79.1%）〕，ventricular wall thickening 〔35 cases（81.4%）〕，myocardial scintillating echo or ground-glass attenuation 〔8 cases（18.6%）〕，restricted diastolic and systolic function 〔21 cases（48.8%）〕，pericardial effusion 〔19 cases（44.2%）〕.Cardiac MRI showed high positive rate （4/4） of late gadolinium enhancement.Among all kinds of biopsies，bone marrow biopsy was the most used〔60.6%（20/33）〕，however，renal biopsy has the highest positive rate（5/6）.（4） Most patients received chemotherapy 〔38 cases（88.4%）〕.The regimens mostly used were BD （bortezomib + dexamethasone） and CyBorD （cyclophosphamide + bortezomib + dexamethasone）.Conclusion　According to the results of this series of cases，the clinical manifestations of patients with AL-CA are very nonspecific.The increase of BNP and cTnI is more common in laboratory tests，electrocardiogram shows low voltage of the limb leads or pathologic Q wave，echocardiography shows concentric left ventricular hypertrophy or myocardial scintillating echo，cardiac MRI shows late gadolinium enhancement.The highest positive results are from renal biopsy among all kinds of biopsies.

Key words: Amyloidosis；Myocardium；Troponin；Echocardiography, stress；Biopsy