Background: Amyloidosis is a disease characterized by the deposition of fibrillar proteins in tissues. The nature of the protein defines the type of amyloidosis. Cardiac involvement is most often secondary to deposits...Background: Amyloidosis is a disease characterized by the deposition of fibrillar proteins in tissues. The nature of the protein defines the type of amyloidosis. Cardiac involvement is most often secondary to deposits of transthyretin and immunoglobulin light chains. Treatment depends on the type of amyloidosis. Cardiac light chain amyloidosis is a medical emergency. Aim: To highlight the importance of an early diagnosis of cardiac light chain amyloidosis. Case Presentation: We report the case of an 88-year-old hypertensive female patient with sustained atrial fibrillation and recurrent heart failure, in whom echocardiography showed concentric left ventricle hypertrophy with mildly reduced left ventricle ejection fraction (LVEF) to 45%. Bone scintigraphy was normal. Serum analysis showed increased lambda free light chains. Accessory salivary gland biopsy revealed weak Kappa light chain staining and clear overexpression of lambda light chain deposits. The diagnosis of stage 3B cardiac amyloidosis secondary to lambda light chain myeloma was made. After a multidisciplinary meeting, it was decided to start treatment with DARATUMUMAB + LENALIDOMIDE. Patient’s general condition deteriorated with the occurrence of febrile pancytopenia. Chemotherapy was stopped and management was limited to comfort care until the patient’s death. Conclusion: Cardiac light-chain amyloidosis must be diagnosed early as it can be rapidly fatal.展开更多
目的探讨尿Kappa轻链(uKAP)、24 h尿蛋白(24 h Pro)、尿Lambda轻链(uLAM)在多发性骨髓瘤(MM)肾功能不全患者中的表达水平及临床意义。方法选取2018年9月至2021年4月在该院诊治的MM患者78例为研究对象。根据预估肾小球滤过率(eGFR)<90...目的探讨尿Kappa轻链(uKAP)、24 h尿蛋白(24 h Pro)、尿Lambda轻链(uLAM)在多发性骨髓瘤(MM)肾功能不全患者中的表达水平及临床意义。方法选取2018年9月至2021年4月在该院诊治的MM患者78例为研究对象。根据预估肾小球滤过率(eGFR)<90 mL/(min·1.73 m^(2))为肾功能不全,eGFR≥90 mL/(min·1.73 m^(2))为肾功能正常将纳入研究的MM患者分为肾功能不全组33例,肾功能正常组45例。检测两组患者肾功能指标[血尿素氮(BUN)、血肌酐(Scr)、视黄醇结合蛋白(RBP)、N-乙酰-β-D-氨基葡萄糖苷酶(NAG)、胱抑素C(Cys C)及血尿酸(UA)]及uKAP、24 h Pro、uLAM水平并进行比较。分析MM患者肾功能指标与uKAP、24 h Pro、uLAM水平的相关性。分析uKAP、24 h Pro、uLAM对MM患者肾功能的影响。结果肾功能不全组BUN、Scr、RBP、NAG、Cys C、UA、uKAP、24 h Pro、uLAM水平均高于肾功能正常组,差异有统计学意义(P<0.05)。Pearson相关分析结果显示,uKAP、24 h Pro、uLAM水平与BUN、Scr、RBP、NAG、Cys C、UA水平均呈正相关(P<0.05)。uKAP、24 h Pro、uLAM水平升高为MM患者肾功能不全的危险因素(P<0.05)。结论uKAP、24 h Pro、uLAM水平在MM肾功能不全患者中呈高表达,是MM患者肾功能不全的危险因素,同时与患者的常规肾功能指标存在相关性。展开更多
文摘Background: Amyloidosis is a disease characterized by the deposition of fibrillar proteins in tissues. The nature of the protein defines the type of amyloidosis. Cardiac involvement is most often secondary to deposits of transthyretin and immunoglobulin light chains. Treatment depends on the type of amyloidosis. Cardiac light chain amyloidosis is a medical emergency. Aim: To highlight the importance of an early diagnosis of cardiac light chain amyloidosis. Case Presentation: We report the case of an 88-year-old hypertensive female patient with sustained atrial fibrillation and recurrent heart failure, in whom echocardiography showed concentric left ventricle hypertrophy with mildly reduced left ventricle ejection fraction (LVEF) to 45%. Bone scintigraphy was normal. Serum analysis showed increased lambda free light chains. Accessory salivary gland biopsy revealed weak Kappa light chain staining and clear overexpression of lambda light chain deposits. The diagnosis of stage 3B cardiac amyloidosis secondary to lambda light chain myeloma was made. After a multidisciplinary meeting, it was decided to start treatment with DARATUMUMAB + LENALIDOMIDE. Patient’s general condition deteriorated with the occurrence of febrile pancytopenia. Chemotherapy was stopped and management was limited to comfort care until the patient’s death. Conclusion: Cardiac light-chain amyloidosis must be diagnosed early as it can be rapidly fatal.
文摘目的探讨尿Kappa轻链(uKAP)、24 h尿蛋白(24 h Pro)、尿Lambda轻链(uLAM)在多发性骨髓瘤(MM)肾功能不全患者中的表达水平及临床意义。方法选取2018年9月至2021年4月在该院诊治的MM患者78例为研究对象。根据预估肾小球滤过率(eGFR)<90 mL/(min·1.73 m^(2))为肾功能不全,eGFR≥90 mL/(min·1.73 m^(2))为肾功能正常将纳入研究的MM患者分为肾功能不全组33例,肾功能正常组45例。检测两组患者肾功能指标[血尿素氮(BUN)、血肌酐(Scr)、视黄醇结合蛋白(RBP)、N-乙酰-β-D-氨基葡萄糖苷酶(NAG)、胱抑素C(Cys C)及血尿酸(UA)]及uKAP、24 h Pro、uLAM水平并进行比较。分析MM患者肾功能指标与uKAP、24 h Pro、uLAM水平的相关性。分析uKAP、24 h Pro、uLAM对MM患者肾功能的影响。结果肾功能不全组BUN、Scr、RBP、NAG、Cys C、UA、uKAP、24 h Pro、uLAM水平均高于肾功能正常组,差异有统计学意义(P<0.05)。Pearson相关分析结果显示,uKAP、24 h Pro、uLAM水平与BUN、Scr、RBP、NAG、Cys C、UA水平均呈正相关(P<0.05)。uKAP、24 h Pro、uLAM水平升高为MM患者肾功能不全的危险因素(P<0.05)。结论uKAP、24 h Pro、uLAM水平在MM肾功能不全患者中呈高表达,是MM患者肾功能不全的危险因素,同时与患者的常规肾功能指标存在相关性。
