获得性血友病A的临床分析与实验室检查

Clinical Analysis and Laboratory Findings in A Patient with Acquired Hemophilia A

为了提高获得性血友病A(acquired hemophilia A)的诊断水平,对1例获得性血友病患者的临床表现、影象学、实验检查结果进行了分析。具体包括了解患者病史,复习静脉彩超记录和分析实验室检查结果。实验室检查项目有:活化部分凝血激酶时间(APTT)、凝血酶原时间(PT)、凝血酶(TT)时间、血浆FⅧ∶C、FⅨ∶C、FⅪ∶C和FⅫ∶C。结果表明:APTT99.3秒,PT13秒,TT13.5秒,FⅧ∶C2(、FⅨ∶C7(、FⅪ∶C9(、FⅫ∶C21(。患者血浆加等量正常新鲜血浆不能使APTT延长完全纠正;患者血浆和等量正常新鲜血浆混合后于37℃温育情况下,APTT随温育时间延长而延长;患者血浆稀释10倍后加等量未稀释正常血浆重复测定结果显示:FⅧ∶C6%、FⅨ∶C75%、FⅪ∶C95(、FⅫ∶C123(。抑制物滴度测定表明:FⅧ抑制物>32Bethesda单位/ml。获得性血友病A确诊后,经强的松、硫唑嘌呤治疗1月余,复查APTT为33.3秒、FⅧ∶C为128(、FⅧ抑制物消失。结论:详细询问病史、分析影象学所见,进行APTT纠正试验、稀释试验和抑制物滴度测定,能减少获得性血友病的误诊和误治,免疫抑制治疗能消除FⅧ抑制物,恢复正常凝血功能。

In order to analyze the clinical features and laboratory findings in patients with acquired hemophilia A, one case of acquired hemophilia A was studied, the medical history, clinical features, ultrasonography and laboratory examination including activated partial thromboplastin time （APTT）, prothrombion time （PT）, thrombin time （TT） and FⅧ： C, FⅣ： C, FⅪ： C, FⅫ： C ratio, as well as medical treatment were analyzed. The results showed 99.3 sec of APTT, 13 sec of PT, and 13.5 sec of TT, 2% of FⅧ： C, 7% of FⅨ： C, 9% of FⅪ： C and 21% of FⅫ： C. The prolongation of APTT could not be completely corrected by mixing the patient plasma with an equal volume of normal fresh plasma; the APTT increased with prolongation of incubation time, when patient plasma was mixed with an equal volume of normal fresh plasma and incubated at 37℃. The plasma FⅧ： C, FⅨ： C, FⅪ： C and FⅫ： C levels in patient were 6%, 75%, 95% and 123% respectively, when patients plasma was diluted by tenfold and mixed with an equal volume of non-diluted normal plasma. FⅧ inhibitor in the patient＇s serum was at a level 〉32.0 Bethesda units/ml after acquired hemophilia was diagnosed, the patient was admited to hospital and given orally prednisone and azathioprin therapy. One month later, clinical status of the patient were improved with 33.3 seconds of APTT, 128% of FⅧ level and elimination of FⅧ inhibitor. In conclusion, inquiring case history, analyzing imaging results, detecting level of AFⅧ, performing dilution test and assaying titer of FⅧ inhibitor can reduce misdiagnosis and wrong therapy for patients with acquined hemophilia A. The FⅧ inhibitor can be eliminated and function of clotting can be recovered by using immunosuppressive therapy.