Park Hyunkyung, Kim Ji-Won, Youk Jeonghwan, Koh Youngil, Lee Jeong-Ok, Kim Ki Hwan, Bang Soo-Mee, Kim Inho, Park Seonyang, Yoon Sung-Soo
Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea; Cancer Research Institute, Seoul National University, Seoul, Korea.
Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Korea.
Clin Lymphoma Myeloma Leuk. 2018 Jun;18(6):408-414. doi: 10.1016/j.clml.2018.03.005. Epub 2018 Mar 15.
AL amyloidosis might increase the risk of thromboembolism and other plasma cell dyscrasias; however, only a few reports have described the clinical features of thromboembolism. The present study aimed to elucidate the clinical features of thromboembolic events and to identify the risk factors for these events.
The medical records were retrospectively reviewed to define the clinically significant thromboembolic events.
A total of 106 patients with biopsy-proven AL amyloidosis were included. During a median follow-up of 18.1 months (range, 0.4-166.9 months), 13 thromboembolism events were identified in 13 patients. Of the 13 patients, 9 (8.5%) experienced acute cerebral infarction, 2 (1.9%) experienced pulmonary embolism, and 2 (1.9%) experienced deep vein thrombosis. Patients with a higher serum free light chain (FLC) difference (≥ 172.4 mg/L) or β-microglobulin (β2MG) levels (≥ 2.78 mg/L) experienced significantly more thromboembolic events compared with those with a lower value according to multivariable analysis (for FLC difference: hazard ratio, 4.309; 95% confidence interval, 1.158-16.032; P = .029; for β2MG: hazard ratio, 9.739; 95% confidence interval, 1.127-84.174; P = .039). Most thromboembolic events (11 of 13; 84.6%) occurred within the first year after the AL amyloidosis diagnosis.
The incidence of thromboembolism was substantial in those with AL amyloidosis. A greater FLC difference and for β2MG levels were risk factors for thromboembolic events.
AL淀粉样变性可能会增加血栓栓塞及其他浆细胞异常增生的风险;然而,仅有少数报告描述了血栓栓塞的临床特征。本研究旨在阐明血栓栓塞事件的临床特征并确定这些事件的危险因素。
对病历进行回顾性分析以确定具有临床意义的血栓栓塞事件。
共纳入106例经活检证实为AL淀粉样变性的患者。在中位随访期18.1个月(范围0.4 - 166.9个月)内,13例患者发生了13次血栓栓塞事件。在这13例患者中,9例(8.5%)发生急性脑梗死,2例(1.9%)发生肺栓塞,2例(1.9%)发生深静脉血栓形成。根据多变量分析,血清游离轻链(FLC)差值较高(≥172.4mg/L)或β2微球蛋白(β2MG)水平较高(≥2.78mg/L)的患者,与数值较低者相比,发生血栓栓塞事件的次数显著更多(对于FLC差值:风险比,4.309;95%置信区间,1.158 - 16.032;P = 0.029;对于β2MG:风险比,9.739;95%置信区间,1.127 - 84.174;P = 0.039)。大多数血栓栓塞事件(13例中的11例;84.6%)发生在AL淀粉样变性诊断后的第一年内。
AL淀粉样变性患者中血栓栓塞的发生率较高。FLC差值增大和β2MG水平升高是血栓栓塞事件的危险因素。