Thông báo lâm sàng trường hợp 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 Hạnh Bùi, Thị Thu Hà Hoàng, Xuân Hùng Lê, Thị Nhâm Nguyễn, Thị Thúy Ngân Nguyễn, Thị Thùy Trang Phan, Minh Hiền Tạ, Thu Huyền Trịnh, Văn Trịnh Vũ

Ngôn ngữ: vie

Ký hiệu phân loại:

Thông tin xuất bản: Y học cộng đồng, 2024

Mô tả vật lý: tr.151-155

Bộ sưu tập: Metadata

ID: 251812

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Coagulation factor V is an important coagulation protein. On activation, factor V play a role of a part of prothrombinase complex which convert prothrombin to thrombin in coagulation cascade. In cell-base model, factor V is able to bind to activated platelets. So, severe factor V deficiency leads to a bleeding tendency. The acquired factor V deficiency usually presents as an autoimmune disorder and it is a rare and challenging codition to treat. It has been associated with major surgeries, antibiotics, blood transfusions, infections, autoimmune disorders, malignancy and exposure to bovine thrombin. Symptoms can range from asymptomatic to life-threatening hemorrhage with mortality rates around 15-20%. Treatment typically involves managing and preventing bleeding episodes through various strategies such as replacement therapy using fresh frozen plasma, immunosuppression therapy or bypass therapy. We describe a case of acquired factor V deficiency after recurrent choledocholithiasis surgery in which the patient developed a postoperative coagulopathy 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.Yếu tố V là một yếu tố đông máu thiết yếu, đóng vai trò như một thành phần của phức hợp prothrombinase nhằm hoạt hóa thrombin, là phản ứng trung tâm của dòng thác đông máu. Trong thuyết đông máu tế bào, yếu tố V còn đóng vai trò hoạt hóa tiểu cầu. Vì vậy, thiếu yếu tố V dẫn đến giảm đông và chảy máu. Thiếu yếu tố V mắc phải là một tình trạng hiếm gặp và khó điều trị, thường do rối loạn tự miễn dịch. Bệnh lý này có liên quan đến các cuộc phẫu thuật lớn, kháng sinh, truyền máu, nhiễm trùng, rối loạn tự miễn dịch, bệnh ác tính và tiếp xúc với thrombin bò. Biểu hiện lâm sàng của thiếu yếu tố V không đồng nhất, có thể không có triệu chứng hoặc chảy máu đe dọa tính mạng với tỷ lệ tử vong từ 15-20%. Nguyên tắc điều trị chủ yếu là kiểm soát chảy máu bằng truyền huyết tương tươi đông lạnh, các liệu pháp ức chế miễn dịch nhằm loại bỏ kháng thể hoặc các liệu pháp bắc cầu.Chúng tôi báo cáo một trường hợp sau mổ sỏi đường mật tái phát có thời gian prothrombin, thời gian prothrombin từng phần hoạt hóa kéo dài được phát hiện thiếu yếu tố V và có kháng thể kháng yếu tố V. Bệnh nhân đã được điều trị thành công bằng liệu pháp ức chế miễn dịch với Steroid và Cyclophosphamide.

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