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頁籤選單縮合
題 名 | 後天血友病之介紹與治療=Introduction and Treatment of Acquired Hemophilia |
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作 者 | 王芙容; 李炳鈺; 王銘崇; 林凱逸; | 書刊名 | 藥學雜誌 |
卷 期 | 28:1=110 2012.03[民101.03] |
頁 次 | 頁114-118 |
分類號 | 415.625 |
關鍵詞 | 後天血友病; 第八凝血因子; 第八凝血因子抑制劑; Acquired hemophilia; Coagulation factor VIII; Coagulation factor VIII inhibitor; |
語 文 | 中文(Chinese) |
中文摘要 | 先天血友病是一種性聯遺傳疾病,因第八或第九凝血因子缺乏所致,病人常在幼年時發病,典型是出現關節出血的問題;後天血友病則是成年人的一種罕見自體免疫疾病,與第八凝血因子被去活化有關,典型症狀與先天血友病不同,主要出現大範圍的表皮紫斑和內部出血。後天血友病的診斷是依據第八凝血因子量過低且血漿有抑制劑存在。治療主要是使用活化態的濃縮 prothrombin 複合體或活化態的第七凝血因子控制出血;使用免疫抑制劑合併類固醇降低抑制劑的產生和增加第八凝血因子的量;rituximab 則是治療後天血友病的新選擇。 |
英文摘要 | Congenital hemophilia is an inherited, sex-linked coagulation disorder due to deficiency of either factor VIII or IX. This is usually associated with bleeding problems from an early age, and bleeding into joints is a typical feature. Acquired hemophilia is a rare condition, and is due to the production of autoantibodies in adult life which inactivate factor VIII. Typical clinical manifestations of the acquired form are extensive cutaneous purpura and internal hemorrhage. Diagnosis is based on the finding of a low factor VIII level associated with the presence of a inhibitor in the plasma. Treatment of the condition involves the use of an activated prothrombin complex concentrate or recombinant activated factor VII to control bleeding episodes, the use of immunosuppression agent with steroids to reduce inhibitor production and bring about a sustained rise in the factor VIII level. Rituximab is a promising new agent in the management of this condition. |
本系統中英文摘要資訊取自各篇刊載內容。