Charaf Edriss, Iskandar Said B, Blevins Ashley, Abi-Saleh Bernard, Fahrig Stephen
Division of Cardiology, University of South Alabama, Mobile, Alabama, USA.
Curr Cardiol Rev. 2009 Aug;5(3):228-36. doi: 10.2174/157340309788970360.
We report a case of a 53-year old patient with symptoms of congestive heart failure in whom a restrictive cardiomyopathy and a kappa-chain monoclonal gammopahty were diagnosed. Treatment with eight cycles of Bortezomib, a proteasome inhibitor, resulted in a significant regression of myocardial amyloid deposition and a notable clinical and hemodynamic improvement. Over the last few years, the management of cardiac amyloidosis has taken advantage of many of the advances of the chemotherapeutic regimens, as well as the wider availability of stem cell transplantation. The management of cardiac amyloidosis is also expected to evolve and improve with the better understanding of the specific mechanisms of amyloidogenesis and myocardial deposition. This will probably make certain molecules targeting specific sites in this process, as potentially effective and minimally toxic compared therapy with the currently used ones. In this article, we describe one of the first reported cases of cardiac amyloidosis, successfully treated with Bortezomib. We describe and discuss the mechanisms of action of Bortezomib and provide a detailed review of cardiac amyloidosis, from pathophysiology to diagnosis and treatment.
我们报告了一例53岁有充血性心力衰竭症状的患者，该患者被诊断为限制性心肌病和κ链单克隆丙种球蛋白病。使用蛋白酶体抑制剂硼替佐米进行八个周期的治疗，使心肌淀粉样蛋白沉积显著消退，并使临床和血液动力学有显著改善。在过去几年中，心脏淀粉样变性的治疗利用了化疗方案的许多进展以及干细胞移植的更广泛应用。随着对淀粉样蛋白生成和心肌沉积的具体机制有了更好的理解，心脏淀粉样变性的治疗也有望得到发展和改善。这可能会使某些针对该过程中特定部位的分子，与目前使用的治疗方法相比，具有潜在的有效性和最小的毒性。在本文中，我们描述了首例成功用硼替佐米治疗的心脏淀粉样变性病例。我们描述并讨论了硼替佐米的作用机制，并对心脏淀粉样变性从病理生理学到诊断和治疗进行了详细综述。
