Abnormal bleeding is sometimes observed in patients with immunoglobulin light chain (AL) amyloidosis. Although several theories have been proposed regarding the pathological causes of the bleeding tendency in AL amyloidosis, many lacked sufficient evidence and full consensus. We conducted a retrospective survey at a single institution to assess bleeding manifestations, methods for evaluating hematological abnormalities, and treatments for bleeding in patients with systemic AL amyloidosis over the past 13 years. The participants were 10 men and 14 women, aged 39-84 years (mean 65 years). The prevalence of bleeding was 29%. Prolonged prothrombin time (PT), elevated plasmin-α2-antiplasmin complex, and factor X deficiency were distinctive to the bleeding group. Two case studies showed that tranexamic acid was effective for treating this hematological condition. However, two patients with normal PT and activated partial thromboplastin time (APTT) also had a bleeding manifestation. The rates of administration of coagulation and fibrinolytic tests were relatively low in the non-bleeding group. Therefore, a close investigation concerning coagulation and fibrinolysis should be performed in every patient with AL amyloidosis regardless of the PT/APTT values. A more careful, comprehensive, and large-scale study is required to reinforce these findings.

中文翻译：

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AL淀粉样变性病的凝血和纤溶特性，具有异常出血和氨甲环酸的实用性。

有时在免疫球蛋白轻链（AL）淀粉样变性患者中观察到异常出血。尽管已经提出了几种有关AL淀粉样变性出血趋势的病理原因的理论，但许多理论缺乏充分的证据和充分的共识。我们在单一机构中进行了回顾性调查，以评估过去13年中系统性AL淀粉样变性患者的出血表现，评估血液学异常的方法以及出血的治疗方法。参与者为10名男性和14名女性，年龄在39-84岁（平均65岁）。出血发生率为29％。凝血酶原时间延长（PT），纤溶酶-α2-抗纤溶酶复合物升高和X因子缺乏是出血组的特征。两个案例研究表明，氨甲环酸有效治疗这种血液病。但是，两名PT正常且部分凝血活酶时间活化（APTT）的患者也有出血表现。在非出血组中，凝血和纤溶测试的给药率相对较低。因此，无论PT / APTT值如何，每位AL淀粉样变性患者均应进行有关凝血和纤维蛋白溶解的密切研究。需要更仔细，全面和大规模的研究以增强这些发现。无论PT / APTT值如何，每位AL淀粉样变性患者均应进行有关凝血和纤维蛋白溶解的密切研究。需要更仔细，全面和大规模的研究以增强这些发现。无论PT / APTT值如何，每位AL淀粉样变性患者均应进行有关凝血和纤维蛋白溶解的密切研究。需要更仔细，全面和大规模的研究以增强这些发现。