PDF(Pubmed)
Abstract：
Systemic amyloid light chain, or primary amyloidosis (AL amyloidosis), is a serious medical condition that leads to the deposition of abnormal proteins called amyloid fibrils in various organs of the body. AL amyloidosis can present with different symptoms, which can make diagnosis challenging. This case report presents a clinical scenario of a 53-year-old female patient who had come in for shortness of breath and lower extremity swelling and was found to have acute on chronic pulmonary embolism. The patient had a history of systemic amyloidosis diagnosed with a kidney and duodenal biopsy. She also had a bone marrow biopsy done and was found to have IgG monoclonal gammopathy. Throughout the hospital course, patients required cautious diuretic use given the worsening kidney function. She was given intravenous anticoagulation initially and later switched to oral medication on discharge. Due to the aggressive nature of amyloidosis, a decision was made to start the patient on chemotherapy in an outpatient setting. This case presents an interesting scenario of systemic amyloidosis with concomitant monoclonal gammopathy that was complicated by acute pulmonary embolism. The case is important as it shows the different levels of amyloidosis and teaches us the benefit of taking a multidisciplinary approach to making a concrete plan for patients with advanced amyloidosis disease.
摘要：
系统性淀粉样蛋白轻链，或原发性淀粉样变性（AL淀粉样变性），是一种严重的医学状况，导致在身体各个器官中沉积称为淀粉样纤维的异常蛋白质。AL淀粉样变性可以表现出不同的症状，这可能使诊断具有挑战性。此病例报告介绍了一名53岁女性患者的临床情况，该患者因呼吸急促和下肢肿胀而被发现患有慢性肺栓塞。该患者有全身性淀粉样变性病史，诊断为肾脏和十二指肠活检。她还进行了骨髓活检，发现患有IgG单克隆丙种球蛋白病。在整个医院过程中，鉴于肾功能恶化，患者需要谨慎使用利尿剂。她最初接受静脉抗凝治疗，后来出院时改用口服药物治疗。由于淀粉样变性的侵袭性，我们决定在门诊患者开始化疗.该病例表现出系统性淀粉样变性伴随有急性肺栓塞并发的单克隆丙种球蛋白病的有趣情况。该病例很重要，因为它显示了淀粉样变性的不同水平，并教会了我们采取多学科方法为晚期淀粉样变性疾病患者制定具体计划的好处。
