Noguchi Hikari, Seki Hiroyuki, Tokumine Joho, Nakazawa Harumasa, Yorozu Tomoko
Anesthesiology, Kyorin University School of Medicine, Tokyo, JPN.
Cureus. 2022 Jul 29;14(7):e27444. doi: 10.7759/cureus.27444. eCollection 2022 Jul.
Acquired hemophilia A is a rare condition caused by autoantibodies against endogenous coagulation factor VIII, which results in spontaneous bleeding. Workup of a patient with difficult hemostasis after removing and placing a central venous catheter led to the diagnosis of acquired hemophilia A. A 64-year-old man was transferred with an intramuscular right thigh mass. Initial biopsy at an outside facility showed degenerated muscle and coagula and he was transferred for incisional biopsy and definitive treatment. The patient had difficult venous access, and a right internal jugular venous catheter was placed. The catheter insertion site showed slow continuous bleeding. Achieving adequate hemostasis after removing the catheter was difficult, and a hematoma formed after the placement of an infraclavicular axillary venous catheter under ultrasound guidance. Coagulation studies revealed a prolonged activated partial thromboplastin time at 96 seconds. The patient was then diagnosed with acquired hemophilia A by enzyme-linked immunosorbent assay using anti-factor VIII antibodies. Even if ultrasound-guided central venous catheterization is performed carefully, bleeding may occur in some patients, suggesting the possibility of coagulopathy. Decision-making for performing central venous catheterization requires extensive knowledge of coagulopathies to understand the causes of bleeding complications.
获得性血友病A是一种由针对内源性凝血因子VIII的自身抗体引起的罕见疾病，可导致自发性出血。对一名在拔除和置入中心静脉导管后止血困难的患者进行检查后，诊断为获得性血友病A。一名64岁男性因右大腿肌内肿块而转诊。外部机构的初步活检显示肌肉退变和凝血块，遂转诊进行切开活检和确定性治疗。该患者静脉穿刺困难，置入了右颈内静脉导管。导管插入部位出现持续缓慢出血。拔除导管后难以实现充分止血，在超声引导下置入锁骨下腋静脉导管后形成了血肿。凝血研究显示活化部分凝血活酶时间延长至96秒。随后通过使用抗因子VIII抗体的酶联免疫吸附试验诊断该患者患有获得性血友病A。即使仔细进行超声引导下的中心静脉置管，某些患者仍可能发生出血，提示存在凝血病的可能性。进行中心静脉置管的决策需要广泛了解凝血病以理解出血并发症的原因。
