Christian Irsara, Andrea Griesmacher, Lorin Loacker, Clemens Feistritzer, Cosima Anna Überbacher, Jean Amiral
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引用次数: 0
摘要
背景:我们报告了一例 59 岁的多发性骨髓瘤患者的病例,该患者体内的抗人凝血酶 IgA 抗体导致体外凝血时间延长,这表明在没有自发性出血的情况下,所有内在凝血因子都受到了抑制:方法:进行常规和大量特殊凝血试验、体内出血时间和特异性抗体检测:结果:虽然患者没有自发性出血,体内出血时间也正常,但抗人类凝血酶 IgA 自身抗体影响了体外所有涉及人类凝血酶的凝血试验,模拟了内在凝血因子的抑制剂。自体干细胞移植后,IgA副蛋白和IgA抗体几乎消失,凝血测试也基本恢复正常:结论:人凝血酶抗体可能会干扰所有涉及凝血酶的凝血检测,从而导致严重的凝血功能障碍。结论:人凝血酶抗体可能会干扰所有涉及凝血酶的凝血测定,模仿严重的凝血病症,但在体内并不一定会导致强烈的出血倾向。复杂和不明确的凝血异常应从一开始就在跨学科的环境中进行评估和治疗,包括高度专业化的凝血实验室。
Anti-Thrombin IgA in a Patient with Multiple Myeloma Leading to In Vitro Interference in Multiple Coagulation Tests and Confounding Diagnosis.
Background: We report the case of a 59-year-old multiple myeloma patient in whom an anti-human thrombin IgA antibody led to prolonged in vitro coagulation times, suggesting inhibitors to all intrinsic coagulation factors in the absence of spontaneous bleeding.
Methods: Routine and extensive special coagulation tests, in vivo bleeding time, and specific antibody testing were performed.
Results: Although the patient did not suffer from spontaneous bleeding and had a normal in vivo bleeding time, the anti-human thrombin IgA autoantibody affected all coagulation assays involving human thrombin in vitro, mimicking inhibitors to intrinsic coagulation factors. As the IgA paraprotein and the IgA antibody virtually disappeared after autologous stem cell transplantation, the coagulation tests also largely normalized.
Conclusion: Antibodies to human thrombin may interfere with all coagulation assays involving thrombin, imitating a severe coagulopathy. However, in vivo they do not necessarily lead to strongly increased bleeding tendency. Complex and ambiguous coagulation abnormalities should be evaluated and treated in an interdisciplinary setting, including a highly specialized coagulation laboratory, from the beginning.
期刊介绍:
Hämostaseologie is an interdisciplinary specialist journal on the complex topics of haemorrhages and thromboembolism and is aimed not only at haematologists, but also at a wide range of specialists from clinic and practice. The readership consequently includes both specialists for internal medicine as well as for surgical diseases.