多发性骨髓瘤致心脏淀粉样变性8例临床分析

Clinical analysis of 8 cases of cardiac amyloidosis due to multiple myeloma

目的探讨多发性骨髓瘤导致的心脏淀粉样变性的临床特征及其预后。方法回顾性分析中国医科大学附属盛京医院及中国医科大学附属第一医院2000—01—01-2012—09—30确诊的8例多发性骨髓瘤（MM）导致的心脏淀粉样变性（CA）病例资料及预后。结果8例患者中男4例，女4例；年龄40～64岁，平均（52．9±7．9）岁。8例患者均因不同程度的胸闷、气促入院，其中伴贫血6例，蛋白尿6例，骨痛3例。8例（100％）均出现多系统淀粉样变性表现，6例典型，2例不典型。且心脏受累均不是首发症状。超声心动图示：室间隔和左室壁均明显增厚伴闪烁颗粒样回声6例。全心呈现细小颗粒样回声2例。病理检查：本组均刚果红（＋），其中，腹壁脂肪4例、舌2例、气管黏膜1例、肾1例。平均随访（16．8±14．6）个月，7例患者死亡。结论MM导致的CA在临床上较少见，但其病情进展快，预后差。对于确诊CA后，同时合并贫血、蛋白尿、骨骼等多系统器官受累时要高度怀疑此病。

Objective To explore the clinical feature and prognosis of cardiac amyloidosis due to multiple myeloma. Meth-ods 8 patients of cardiac amyloidosis due to multiple myeloma were analyzed and followed up. Results Eight patients,4 men and 4 women, with mean age of 40 - 64 （ 52. 9 ± 7.9 ） years older were enrolled. The causes of admission in the pa-tients were almost the same, due to different degree of dyspnea and taehypnea. Anaemia, proteinuria and bone pain were 75% ,75% and 37. 5% , respectively in 8 patients. For amyloid deposition,8 patients （ 100% ） had involvement in more than one organ. Among the 8 patients,6 developed typical features ,2 had atypical features, in addition, none of the 8 patients with initial presentation of cardiac involvement. UCG showed that 6 patients had a thickened left ventricle with a granular speckled appearance. 2 patients had the diffuse granular sparkling appearance of the whole cardiac structure. Followed up for （ 16. 8 ± 14. 6） months, seven of the patients （ 87.5% ） died. Pathological： congo red staining was positive in all the pa-tient： The biopsy of abdominal fat in 4 patients, the biopsy of tongue tissue in 2 patients, the biopsy of tracheal mucous in 1 patient and the biopsy of kindey in 1 patient. Conclusion Cardiac due to multiple myeloma is rare clinically,but has fast aggravation and bad prognosis. Lenalidomide is an effective therapeutic drugs on the control of the underlying multiple mye-loma and amyloidosis.