心脏原发轻链型淀粉样变1例报告并文献复习

Report of cardiac primary light-chain amyloidosis in one case and literature review

目的探讨心脏原发轻链型淀粉样变临床特点、病理特征、诊断及治疗。方法报道1例心脏原发轻链型淀粉样并复习相关文献。结果心脏原发轻链型淀粉样变临床特点包括常见心力衰竭症状,顽固性低血压,心电图多有低电压,异常Q波等表现;超声心动图特异性表现为心内膜心肌呈现出闪耀的颗粒状;单克隆轻链异常增高;组织活检,经刚果红染色可见特异性淀粉样组织。常用治疗方法为MP或MD方案(美法仑+泼尼松或地塞米松)。结论心脏原发轻链型淀粉样变临床罕见,特异性临床表现少,组织活检、检测血清游离轻链为主要确诊手段;治疗为MP或MD方案。

Objective To discuss the clinical features, pathological characteristics, diagnosis, and treatment of cardiac primary light - chain amyloidosis. Methods A case of cardiac primary light - chain amyloidosis was reported, and relative literatures were reviewed. Results The main clinical features included heart failure, refractory hypotension, and low voltage and abnormal Q wave on the electrocardiogram, etc. The specificity of the ultrasonic cardiogram included the occurrence of particles in the cardiac muscle of endocardium and abnormal increase of monoclonal light chain. In the tissue biopsy, specific amyloidosis tissues could be observed after the staining by Congo red. The conventional treatment methods included MP or MD scheme （melphalan plus prednisone or dexamethasone ）. Conclusion Cardiac primary light - chain amyloidosis is rarely seen in clinic, and there are few specific clinical manifestations. Tissue biopsy and serum free light chain detection are the main methods for the final diagnosis, and MP and MD schemes are the treatment methods.