关键词: Haematology (incl blood transfusion) Thrombosis

Mesh : Humans Male Venous Thrombosis / etiology diagnosis Factor X Deficiency / diagnosis complications Middle Aged Immunoglobulin Light-chain Amyloidosis / complications diagnosis

来  源:   DOI:10.1136/bcr-2024-260169

Abstract:
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.
摘要:
获得性因子X(FX)缺乏是AL淀粉样变性的罕见但有据可查的临床特征。由于循环FX吸附到淀粉样原纤维上,FX缺乏症患者可表现出临床上显着的出血素质。这里,我们报道了一个不寻常的病例,一个60多岁的男人出现了6个月的间歇性瘀伤,实验室证明了新的外汇缺陷,游离λ轻链升高用于潜在的AL淀粉样变性和并发的新的腓骨静脉血栓形成。这是关于AL-淀粉样蛋白诱导的FX缺乏症中并发血栓并发症的首次报道。我们讨论了在FX缺乏的情况下,以瘀伤为主要临床症状的AL淀粉样变性的诊断和治疗难题,以及急性深静脉血栓形成的管理。
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