Báo cáo ca lâm sàng thiếu yếu tố V mắc phải sau mổ sỏi đường mật tái phát điều trị thành công với thuốc ức chế miễn dịch=Clinical case report - the acquired factor V deficiency after recurrent choledocholithiasis surgery, successfully treated with immunosuppressive therapy

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Tác giả: Thị Thiết Đào, Thị Thu Hà Hoàng, Thị Mai Nguyễn, Thị Thuỳ Trang Phan, Thị Quyên Trần

Ngôn ngữ: vie

Ký hiệu phân loại:

Thông tin xuất bản: Tạp chí Y học Việt Nam (Tổng hội Y học Việt Nam), 2024

Mô tả vật lý: tr.714-721

Bộ sưu tập: Báo, Tạp chí

ID: 478353

Thêm vào giỏ

Factor V deficiency is also known as Owren’s disease or parahemophilia. It’s a rare bleeding disorder, occurring in only one in one million people. It can manifest as either congenital or acquired. The acquired factor V deficiency usually presents as an autoimmune disorder. Symptoms can range from asymptomatic to life-threatening hemorrhage with mortality rates around 15-20%. Subjects and method: We describe a case of acquired factor V deficiency after recurrent choledocholithiasis surgery in which the patient developed a postoperative bleed with prolonged prothrombin time and active partial thromboplastin time associated with reduced plasma factor V content and factor V inhibitor titers. The patient was successfully cured by immunosuppressive therapy including steroids and cyclophosphamide. Results: To our knowledge, this is the first case of FV inhibitor antibodies developing after recurrent choledocholithiasis surgery in Viet Nam. While a rare phenomenon, it is important for hematologists to be aware of such a case and the potential serious clinical implications.Thiếu yếu tố V mắc phải là một tình trạng hiếm gặp và thách thức trong chẩn đoán điều trị. Biểu hiện lâm sàng của thiếu yếu tố V có thể không đồng nhất, từ trạng thái lâm sàng không có triệu chứng với các xét nghiệm đông máu bất thường đến chảy máu đe dọa tính mạng. Mặc dù sự thiếu hụt yếu tố V có thể gặp bẩm sinh hoặc mắc phải nhưng trong thực hành lâm sàng thiếu yếu tố V mắc phải do rối loạn tự miễn dịch thường gặp hơn cả. Đối tượng và phương pháp nghiên cứu: Báo cáo một trường hợp nam giới, 65 tuổi, sau mổ sỏi đường mật tái phát có thời gian prothrombin và thromboplastin từng phần hoạt hóa (aPTT) kéo dài, phát hiện thiếu hụt yếu tố V và kháng thể kháng yếu tố V dương tính. Bệnh nhân đã được điều trị thành công bằng liệu pháp ức chế miễn dịch bao gồm steroid và cyclophosphamide. Kết quả: Theo hiểu biết của chúng tôi đây là trường hợp thiếu yếu tố V mắc phải đầu tiên được báo cáo ở Việt Nam. Báo cáo đóng góp thêm dữ liệu về triệu chứng lâm sàng và xét nghiệm cũng như kinh nghiệm điều trị thiếu yếu tố V mắc phải

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