威尔逊氏病患者发生DIC的罕见病例:d -青霉胺。

IF 1.2 Q4 GASTROENTEROLOGY & HEPATOLOGY Hepatology Forum Pub Date : 2022-04-26 eCollection Date: 2022-05-01 DOI:10.14744/hf.2022.2022.0001
Yavuz Emre Parlar, Hatice Yasemin Balaban, Umit Yavuz Malkan, Erdogan Deniz, Fatma Rukiye Uysal
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引用次数: 0

摘要

d -青霉胺治疗被认为是一种有效和安全的治疗威尔逊氏病的方法。除了一项实验研究外,文献中没有关于使用该药会发生弥散性血管内凝血(DIC)的报道。一名24岁女性Wilson病患者,经锌和d -青霉胺治疗后,因口腔黏膜出血和嗜睡而入院急诊。初步实验室检查显示血红蛋白7.1 g/dL(11.7-15.5),血小板24×103µL-1(159-388),总胆红素18 mg/dL(0.3-1.2),直接胆红素9.8 mg/dL (0-0.2), INR >10 (0.8-1.2), aPTT 64.5 s(22.5-32),纤维蛋白原23 mg/dL(180-350),因子8 26.4%(70-150)。未见黑黑、呕血、便血,内窥镜检查未见活动性出血灶。在达到DIC标准后,患者接受了四次血浆置换治疗急性慢性肝衰竭。全血蛋白p、低温沉淀置换作为DIC的支持治疗。尽管进行了血浆置换和因子替代治疗,但临床出血仍在继续,因此将d -青霉胺改为曲恩汀(1250 mg/天)。改变后,粘膜出血停止,DIC参数改善。我们建议,如果d -青霉胺治疗后出现出血并发症,还应考虑d -青霉胺激活纤维蛋白溶解诱导DIC的可能性。
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A rare case of DIC in a patient with Wilson's disease: D-penicillamine.

D-penicillamine therapy is considered an effective and safe treatment for Wilson's disease. Except for one experimental study, there has been no report in the literature about the development of disseminated intravascular coagulation (DIC) with the use of the drug. A 24-year-old female patient with Wilson's disease, followed up with zinc and D-penicillamine treatment, was admitted to the emergency service because of oral mucosal bleeding and lethargy. Initial laboratory tests showed hemoglobin 7.1 g/dL (11.7-15.5), platelet 24×103 µL-1 (159-388), total bilirubin 18 mg/dL (0.3-1.2), direct bilirubin 9.8 mg/dL (0-0.2), INR >10 (0.8-1.2), aPTT 64.5 s (22.5-32), fibrinogen 23 mg/dL (180-350), and factor 8 26.4% (70-150). Melena, hematemesis, and hematochezia were not present, and no active bleeding focus was detected on endoscopic evaluation. Upon meeting the DIC criteria, the patient underwent plasma exchange four times for the treatment of acute-on-chronic liver failure. Haemocomplettan-P, cryoprecipitate replacements were made as a supportive treatment for DIC. As the clinical bleeding continued despite plasma exchanges and factor replacement treatment, D-penicillamine was switched to trientine (1250 mg/day). After this change, the mucosal bleeding stopped, and DIC parameters improved. We suggest that if hemorrhagic complications develop on D-penicillamine treatment, the possibility of DIC induced by D-penicillamine activating the fibrinolysis should also be considered.

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