充血性心力衰竭（CHF）是AL淀粉样变性患者的常见并发症，但在另一种浆细胞发育不良中很少见。POEMS综合征。一名52岁的男子患有POEMS综合征，伴有孤立性浆细胞瘤，并伴有CHF，模仿心脏淀粉样变性（CA）。针对浆细胞瘤的放疗（50Gy）后，他的神经系统症状和CHF没有改善。基于非侵入性检查的典型结果，例如血清NT-proBNP升高（12,631pg/mL），心电图上的假性梗塞模式,室间隔增厚，在超声心动图上表现为颗粒状闪闪发光的外观和根尖保留的纵向应变模式，和心脏磁共振成像（MRI）的左心室壁的晚期钆增强，强烈怀疑不适合自体外周血干细胞移植(auto-PBSCT)的重度CA.然而,心肌活检未能发现淀粉样蛋白沉积物,仅使用美法仑和地塞米松进行一个周期的化疗后，CHF显着改善。因此，CA被否认为心力衰竭的病因,患者最终被诊断为POEMS综合征。因此,大剂量美法仑和auto-PBSCT改善了他的神经症状.因此，需要仔细评估以适当治疗POEMS综合征并发CHF的患者，即使非侵入性检查的结果是典型的AL淀粉样变性。
Congestive heart failure (CHF) is a common complication in patients with AL amyloidosis but is rare in another plasma cell dyscrasia, POEMS syndrome. A 52-year-old man developed POEMS syndrome with a solitary plasmacytoma complicated by CHF mimicking cardiac amyloidosis (CA). His neurological symptoms and CHF did not improve after radiotherapy (50 Gy) targeting the plasmacytoma. Based on typical findings of noninvasive examinations such as elevated serum NT-proBNP (12,631 pg/mL), a pseudo-infarct pattern on electrocardiography, interventricular septal thickening with a granular sparkling appearance and an apical sparing pattern of longitudinal strain on echocardiography, and late gadolinium enhancement of the left ventricular wall on cardiac magnetic resonance imaging (MRI), severe CA ineligible for autologous peripheral blood stem cell transplantation (auto-PBSCT) was strongly suspected. However, myocardial biopsy failed to reveal amyloid deposits, and CHF markedly improved after only one cycle of chemotherapy with melphalan and dexamethasone. Accordingly, CA was denied as the etiology of his heart failure, and the patient was finally diagnosed with POEMS syndrome. As a result, high-dose melphalan followed by auto-PBSCT improved his neurological symptoms. Careful evaluation is therefore needed to appropriately treat patients with POEMS syndrome complicated by CHF, even when the results of non-invasive examinations are typical for AL amyloidosis.
PDF(Pubmed)