Kalluri Sneha, Abbasi Jamil
Internal Medicine, Baylor Scott & White All-Saints Medical Center, Fort Worth, USA.
Critical Care, Baylor Scott & White All-Saints Medical Center, Fort Worth, USA.
Cureus. 2023 Jun 19;15(6):e40658. doi: 10.7759/cureus.40658. eCollection 2023 Jun.
Amyloidosis is a plasma cell dyscrasia that leads to the excessive production and deposition of mutant protein fragments in various organs. Cardiac amyloidosis is often implicated in two main subtypes: transthyretin (ATTR) and light chain (AL). While both subtypes increase the risk of restrictive cardiomyopathy, cardiogenic shock, and arrhythmias, poorer outcomes are seen in those with cardiac infiltration secondary to AL amyloidosis. Prognosis depends on the timing of diagnosis and the extent of the disease burden prior to recognition and treatment. The following case report describes a young patient who was admitted to the intensive care unit (ICU) for concerns of decompensated heart failure of unknown etiology, later determined to be due to amyloidosis. We describe her clinical course prior to and during hospital admission, along with the proposed physiologic factors that may have contributed to her poor outcome.
淀粉样变性是一种浆细胞发育异常，可导致突变蛋白片段在各个器官中过度产生和沉积。心脏淀粉样变性通常涉及两种主要亚型：转甲状腺素蛋白（ATTR）和轻链（AL）。虽然这两种亚型都会增加限制性心肌病、心源性休克和心律失常的风险，但继发于AL淀粉样变性的心脏浸润患者的预后较差。预后取决于诊断时间以及在识别和治疗之前疾病负担的程度。以下病例报告描述了一名年轻患者，因不明病因的失代偿性心力衰竭而入住重症监护病房（ICU），后来确定是由淀粉样变性引起的。我们描述了她入院前和住院期间的临床过程，以及可能导致她预后不良的推测生理因素。
