Department of Medicine, Oregon Health & Science University, Portland, Oregon, USA.
Department of Medicine, Division of Hematology & Medical Oncology, Oregon Health & Science University Knight Cancer Institute, Portland, Oregon, USA.
BMJ Case Rep. 2024 Jun 5;17(6):e260169. doi: 10.1136/bcr-2024-260169.
Acquired factor X (FX) deficiency is a rare but well-documented clinical feature of AL amyloidosis. Patients with FX deficiency can present with clinically significant bleeding diathesis due to the adsorption of circulating FX to amyloid fibrils. Here, we report an unusual case of a man in his 60s who presented with 6 months of intermittent bruising, labs demonstrating new FX deficiency, elevated free lambda light chains for underlying AL amyloidosis and concurrent new peroneal vein thrombosis. This is the first report of concurrent thrombotic complications in the setting of AL-amyloid-induced FX deficiency. We discuss the diagnostic and therapeutic conundrum of diagnosing AL amyloidosis with bruising as the leading clinical symptom and the management of acute deep vein thrombosis in the setting of FX deficiency.
获得性因子 X (FX) 缺乏症是 AL 淀粉样变性的一种罕见但有充分文献记录的临床特征。由于循环 FX 被吸附到淀粉样纤维上,FX 缺乏症患者可能会出现具有临床意义的出血倾向。在此,我们报告了一例不常见的 60 多岁男性病例,他出现了 6 个月的间歇性瘀斑,实验室检查显示新的 FX 缺乏症、游离 λ 轻链升高提示潜在的 AL 淀粉样变性和同时发生的新腓静脉血栓形成。这是首例报告的在 AL 淀粉样蛋白诱导的 FX 缺乏症的背景下同时发生血栓并发症的病例。我们讨论了以瘀斑为主要临床症状的 AL 淀粉样变性的诊断和治疗难题,以及 FX 缺乏症背景下急性深静脉血栓形成的处理。