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[一名患有类固醇抵抗性获得性血友病A和肾病综合征的患者在早期胃癌切除术后实现凝血完全缓解]

[Coagulative complete remission following early gastric cancer resection in a patient with steroid-resistant acquired hemophilia A and nephrotic syndrome].

作者信息

Matsumoto Akira, Ogawa Yoshiyuki, Osaki Tsukasa, Souri Masayoshi, Takei Hisashi, Ishikawa Tetsuya, Kobayashi Nobuhiko, Miyazawa Yuri, Ishizaki Takuma, Inoue Madoka, Ichinose Akitada, Handa Hiroshi

机构信息

Department of Hematology, Gunma University Graduate School of Medicine.

Department of Public Health and Hygiene, Yamagata University Faculty of Medicine.

出版信息

Rinsho Ketsueki. 2023;64(3):203-208. doi: 10.11406/rinketsu.64.203.

DOI:10.11406/rinketsu.64.203
PMID:37019674
Abstract

During laparoscopic cholecystectomy, an 89-year-old man was discovered to have a prolonged APTT. He was transferred to our hospital for a thorough examination because wound bleeding necessitated a reoperation. Based on coagulation factor VIII activity (FVIII:C) of 3.6% and FVIII inhibitor levels of 48.5 BU/ml, he was diagnosed with acquired hemophilia A (AHA). Due to concerns about his advanced age and postoperative infection, immunosuppressive therapy with prednisolone 0.5 mg/kg/day was initiated. His clinical course was favorable, except hemorrhagic shock caused by intramuscular hemorrhage on the right back, although low FVIII inhibitor levels persisted for more than a month; additionally, lower leg edema and increased urinary protein were also observed. He was diagnosed as with AHA and secondary nephrotic syndrome, possibly because of early gastric cancer. As a result, radical endoscopic submucosal dissection (ESD) was performed while a recombinant coagulation factor VIIa preparation was administered. AHA improved rapidly following ESD, and coagulative remission was achieved. Simultaneously, the nephrotic syndrome improved. Because the control of malignant tumors may improve the status of AHA, the timing of malignant tumor intervention must be considered considering the risk of bleeding and infection associated with immunosuppression.

摘要

在腹腔镜胆囊切除术期间,发现一名89岁男性的活化部分凝血活酶时间(APTT)延长。由于伤口出血需要再次手术,他被转至我院进行全面检查。根据凝血因子VIII活性(FVIII:C)为3.6%以及FVIII抑制物水平为48.5 BU/ml,他被诊断为获得性血友病A(AHA)。鉴于其高龄及术后感染风险,开始使用泼尼松龙0.5 mg/kg/天进行免疫抑制治疗。他的临床过程较为顺利,除了右背部肌肉出血导致的失血性休克外,尽管低FVIII抑制物水平持续了一个多月;此外,还观察到小腿水肿和尿蛋白增加。他被诊断为患有AHA和继发性肾病综合征,可能是由于早期胃癌所致。因此,在给予重组凝血因子VIIa制剂的同时,进行了根治性内镜黏膜下剥离术(ESD)。ESD后AHA迅速改善,并实现了凝血缓解。同时,肾病综合征也有所改善。由于控制恶性肿瘤可能改善AHA的状况,因此必须考虑恶性肿瘤干预的时机,同时要考虑免疫抑制相关的出血和感染风险。

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