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心肌淀粉样变性的临床病理

毛歆歆,  赵大春,  方全,  田庄,  程中伟,  崔全才

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文章导航 > 协和医学杂志  > 2012 >  3(1): 89-94
毛歆歆, 赵大春, 方全, 田庄, 程中伟, 崔全才. 心肌淀粉样变性的临床病理[J]. 协和医学杂志, 2012, 3(1): 89-94. doi: 10.3969/j.issn.1674-9081.2012.01.019
引用本文: 毛歆歆, 赵大春, 方全, 田庄, 程中伟, 崔全才. 心肌淀粉样变性的临床病理[J]. 协和医学杂志, 2012, 3(1): 89-94. doi: 10.3969/j.issn.1674-9081.2012.01.019
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Xin-xin MAO, Da-chun ZHAO, Quan FANG, Zhuang TIAN, Zhong-wei CHENG, Quan-cai CUI. Clinicopathological Features of Cardiac Amyloidosis[J]. Medical Journal of Peking Union Medical College Hospital, 2012, 3(1): 89-94. doi: 10.3969/j.issn.1674-9081.2012.01.019
Citation: Xin-xin MAO, Da-chun ZHAO, Quan FANG, Zhuang TIAN, Zhong-wei CHENG, Quan-cai CUI. Clinicopathological Features of Cardiac Amyloidosis[J]. Medical Journal of Peking Union Medical College Hospital, 2012, 3(1): 89-94. doi: 10.3969/j.issn.1674-9081.2012.01.019
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心肌淀粉样变性的临床病理

doi: 10.3969/j.issn.1674-9081.2012.01.019
  • 1.

    中国医学科学院 北京协和医学院 北京协和医院 病理科, 北京 100730

  • 2.

    中国医学科学院 北京协和医学院 北京协和医院 心内科, 北京 100730

详细信息
    通讯作者:

    崔全才 电话:010-65295520, E-mail:cuiqc@sina.com

  • 中图分类号: R542.2;R446.8

Clinicopathological Features of Cardiac Amyloidosis

  • 1.

    Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China

  • 2.

    Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China

More Information
    Corresponding author: CUI Quan-cai Tel: 010-65295520, E-mail:cuiqc@sina.com

    摘要
  • 摘要:   目的  探讨心肌淀粉样变的临床病理特征、诊断及治疗。  方法  回顾性分析北京协和医院2006年9月至2010年4月63例心肌活检病例, 其中17例经病理组织活检证实为心肌淀粉样变, 分析其临床特征、诊断及治疗并复习文献。  结果  17例心肌淀粉样变患者中(1)82.4%发病年龄> 40岁, 男女比例1:1;94%(16/17)有2个或以上系统受累, 3个系统以上受累占52.9%(9/17);一般表现乏力者占41%(7/17)、皮肤瘀点瘀斑占23.5%(4/17)。(2)循环系统呈现低血压趋势患者占76.5%;超声心动图检查左室后壁厚(14.6±2.27)mm、室间隔或左室壁厚度> 1.1 mm者占82.4%(14/17), 82.4%(14/17)的患者有左室舒张功能受损表现, 76.5%(13/17)的患者发现颗粒样心肌回声增强; 心电图异常者占82.4%(14/17)。(3)消化系统症状达47%(8/17), 舌体肥大者占11.7%(2/17), 肝脏肿大者占17.6%(3/17)。(4)41.2%(7/17)的患者表现为肾功能不全。(5)52.9%(9/17)的患者血和尿免疫固定电泳(immunofixation electrophoresis, IFE)发现单克隆免疫球蛋白轻链, 其中λ轻链8例, κ轻链1例。(6)5.9%(1/17)患者出现神经系统表现。  结论  心肌淀粉样变呈多系统受累, 心脏、消化系统、肾脏为常见的靶器官, 本病预后不良, 早期诊断尤为重要。
    Abstract:   Objective  To explore the clinical features of cardiac amyloidosis (CA).  Methods  The clinical data of 17 CA patients who were diagnosed in our hospital from September 2006 to April 2010 were retrospectively analyzed.  Results  Of these 17 patients, 14 (82.4%) were over 40 years old, and the ratio of male to female was 1:1. Sixteen patients (94%) had two or more systems involved, and 9 patients (52.9%) had three or more systems involved. Patients were manifested as fatigue (41%) and/or skin petechia/ecchymosis. In the circulation system, seven patients (41.2%) showed a trend of hypotension, and echocardiography showed 14 patients (82.4%) had thickened ventricular walls[(14.6±2.27) mm]and interventricular septal thickness/left ventricular wall thickness >1.1 mm. Fourteen patients (82.4%) had impaired diastolic function of ventriculus sinister. Thirteen patients (76.5%) had increased echogenicity of the myocardium with a granular appearance. Fourteen patients (82.4%) had abnormal electrocardiogram findings. Eight patients (47%) had gastrointestinal systems, which included glossal hypertrophy (11.7%) and enlargement of the liver (17.6%). Seven patients (41.2%) showed renal insufficiency. Immunofixation electrophoresis of blood and urine detected monoclonal light chain in 52.9% of patients (λ light chain, n=8; κ light chain, n=1). One patient (5.9%) experienced nervous symptom.  Conclusion  CA can affect multiple systems, with heart, gastrointestinal system, and kidney as the most commonly involved target organs.
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  • 图  1  光镜下淀粉样物质沉积于心肌间质呈粉红色

    下载: 全尺寸图片 幻灯片

    图  2  刚果红染色淀粉样物质呈砖红色

    下载: 全尺寸图片 幻灯片

    图  3  淀粉样物质在偏光显微镜下呈苹果绿色

    下载: 全尺寸图片 幻灯片

    图  4  患者, 男性, 53岁, 超声心动图表现为室间隔及左室后壁明显增厚, 回声增强, 左房扩大, 左室腔略缩小(A); 心电图表现为I度房室传导阻滞, ST-T段改变(B)

    下载: 全尺寸图片 幻灯片

    表  1  心肌淀粉样变17例患者系统受累情况
    • 参考文献(16)
  • [1] Shirahama T, Cohen AS. High-resolution electron microscopic analysis of the amyloid fibril[J]. J Cell Biol, 1967, 33:679-708. doi:  10.1083/jcb.33.3.679
    [2] Westermark P. Aspects on human amyloid forms and their fibril polypeptides[J]. FEBS J, 2005, 272:5942-5949. doi:  10.1111/j.1742-4658.2005.05024.x
    [3] Levy DJ, Franklin GO, Rosenthal WS. Gastrointestinalbleeding and amyloidosis[J]. Am J Gastroenterol, 1982, 77:422-426. http://europepmc.org/abstract/MED/6979925
    [4] Wu D, Lou JY, Chen J, et al. A case report of localizedgastric amyloidosis[J]. World Gastroentoerl, 2003, 9:2632-2634. doi:  10.3748/wjg.v9.i11.2632
    [5] Furie B, Vool L, MeAdam KP, et al. Mechanism of factorX deficiency in systemic amyloidosis[J]. N Engl J Med, 1981, 304:827-830. doi:  10.1056/NEJM198104023041407
    [6] 钟淑萍.原发性淀粉样变并发凝血因子Ⅶ缺乏[J].中国实用医药, 2008, 18:39-40. http://www.wanfangdata.com.cn/details/detail.do?_type=perio&id=zgsyyy200818018
    [7] Goldman L, Ausiello D. Cecil textbook of medicine[M]. 22ed. Philadelphia: Saunders, 2004: 17132-17163.
    [8] Ebert EC, Nagar M. Gastrointestinal manifestations ofamyloidosis[J]. Am J Gastroenterol, 2008, 103:776-787. doi:  10.1111/j.1572-0241.2007.01669.x
    [9] Pollak A, Falk RH. Left ventricular systolic dysfunction precipitated by verapamil in cardiac amyloidosis[J]. Chest, 1993, 104:618-620. doi:  10.1378/chest.104.2.618
    [10] Mignot A, Varnous S, Redonnet M, et al. Heart transplantation in systemic (AL) amyloidosis:a retrospective study of eight French patients[J]. Arch Cardiovasc Dis, 2008, 101:523-532. doi:  10.1016/j.acvd.2008.06.018
    [11] Kristen AV, Sack FU, Schonland SO, et al. Staged heart transplantation and chemotherapy as a treatment option in patients with severe cardiac light-chain amyloidosis[J]. Eur J Heart Fail, 2009, 11:1014-1020. doi:  10.1093/eurjhf/hfp121
    [12] Hosenpud JD, Uretsky BF, Griffith BP, et al. Successful intermediate-term outcome for patients with cardiac amyloidosis undergoing heart transplantation:results of a multicenter survey[J]. J Heart Transplant, 1990, 9:346-350. http://europepmc.org/abstract/MED/2398427
    [13] Mignot A, Bridoux F, Thierry A, et al. Successful heart transplantation following melphalan plus dexamethasone therapy in systemic AL amyloidosis[J]. Haematologica, 2008, 93:e32-e35. doi:  10.3324/haematol.12108
    [14] Palladini G, Lavatelli F, Russo P, et al. Circulating amyloidogenic free light chains and serum N-terminal natriuretic peptide type B decrease simultaneously in association with improvement of survival in AL[J]. Blood, 2006, 107:3854-3858. doi:  10.1182/blood-2005-11-4385
    [15] Audard V, Matignon M, Weiss L, et al. Successful longterm outcome of the first combined heart and kidney transplant in a patient with systemic AL amyloidosis[J]. Am J Transplant, 2009, 9:236-240.
    [16] Skinner M, Sanchorawala V, Seldin DC, et al. High-dose melphalan and autologous stem-celltransplantation in patients with AL amyloidosis:an 8-year study[J]. Ann Intern Med, 2004, 40:85-93. http://www.ncbi.nlm.nih.gov/pubmed/14734330
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出版历程
  • 收稿日期:  2011-11-08
  • 刊出日期:  2012-01-30

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