Yaghubi Mohsen, Dinpanah Hossein, Ghanei-Motlagh Fahimeh, Kakhki Samaneh, Ghasemi Reza
MSc Student, Student Research Committee AND Department of Cardiac Surgery, Mashhad University of Medical Sciences, Mashhad, Iran.
Assistant Professor, Department of Emergency, Dey 9th Hospital, Torbat Heydariyeh University of Medical Sciences, Torbat Heydariyeh, Iran.
ARYA Atheroscler. 2018 Mar;14(2):101-104. doi: 10.22122/arya.v14i2.1676.
Amyloidosis is a severe systemic disorder produces by the accumulation of inappropriately amyloid deposition in tissues. Cardiac involvement, as a main type of amyloidosis, has a major impact on prognosis. We describe a biopsy-proven cardiac amyloidosis in an old man with unexpected presentation.
A 70-year-old man, with a complaint of severe weakness, lightheadedness, and lower limb paresthesia, was admitted to the emergency department. Electrocardiography revealed right bundle branch block and Trifascicular block. Echocardiography study showed a moderately increased thickness of left ventricular wall with concentric pattern as well. Laboratory investigations including serum and urine electrophoresis, and serum free light chain examination as immunofixation assay revealed that κ chains predominated over λ chains in a ratio of 3:2. Our patient with final diagnosis of amyloid light-chain (AL) amyloidosis underwent chemotherapy with melphalan combined with high-dose dexamethasone, CPHPC and monoclonal antibodies for 2 weeks.
It shows that rapid diagnosis of AL amyloidosis can enhance the prognosis. Applying an optimal strategy for the treatment leads to effective therapy, too.
淀粉样变性是一种严重的全身性疾病,由组织中不适当的淀粉样蛋白沉积积累所致。心脏受累作为淀粉样变性的主要类型,对预后有重大影响。我们描述了一例经活检证实的老年心脏淀粉样变性患者,其表现出人意料。
一名70岁男性,因严重乏力、头晕和下肢感觉异常入院至急诊科。心电图显示右束支传导阻滞和三分支阻滞。超声心动图检查显示左心室壁厚度中度增加,呈同心模式。包括血清和尿液电泳以及血清游离轻链检查(免疫固定测定)在内的实验室检查显示,κ链与λ链的比例为3:2,κ链占优势。最终诊断为轻链型(AL)淀粉样变性的患者接受了美法仑联合大剂量地塞米松、CPHPC和单克隆抗体化疗2周。
这表明AL淀粉样变性的快速诊断可改善预后。应用最佳治疗策略也可实现有效治疗。