CAJ | 학술논문

目的探讨获得性血友病A（ AHA）的临床特点、诊断与治疗方法，以提高对本病的认识。方法对25例获得性血友病A患者的临床表现、实验室检查、诊治经过与疗效等临床资料进行分析。结果25例患者中，男13例，女12例；年龄53．28±19．78岁；11例发病前无基础疾病及明确诱因，病程3 d～2年。临床表现除基础疾病表现外，均有不同程度的皮肤瘀斑、肌肉肿胀疼痛、牙龈渗血、血尿、血便、腹腔血肿等出血表现；实验室检查为重要的依据，表现为aPTT延长，为94．9±20．9秒，血浆混合试验aPTT不能被正常血浆纠正，FⅧ：C降低为4．5±6．7％，FⅧ抑制物滴度在22．4±24．3 Bu；以上病例治疗根据出血情况及基础疾病与全身状况，予止血、消除抗体措施，获得完全缓解19例，部分缓解5例，死亡1例。其中5例使用利妥昔单抗治疗的患者均获得完全缓解。获缓解的24例，按FⅧ抑制物滴度水平分为3组，其与出血的严重程度、aPTT、FⅧ：C、获得部分缓解的时间等因素进行分析，显示并无明确的相关性。上述病例经治疗后临床症状好转，FⅧ活性升高至50％时间为23．8±13．3 d，FⅧ活性恢复正常时间为86．2±76．8 d；FⅧ抑制物滴度减少50％的时间28±16．7 d，FⅧ抑制物滴度≤0．6 Bu或转阴时间156±230．5 d。结论以上病例经及早诊断、及时补充凝血因子与应用免疫抑制药物后，出血症状可明显减轻，病情缓解，但要FⅧ抗体转阴则需较长时间治疗及结合其他疗法，利妥昔单抗联合免疫治疗已取得较好疗效，值得进一步研究。
목적탐토획득성혈우병A（ AHA）적림상특점、진단여치료방법，이제고대본병적인식。방법대25례획득성혈우병A환자적림상표현、실험실검사、진치경과여료효등림상자료진행분석。결과25례환자중，남13례，녀12례；년령53．28±19．78세；11례발병전무기출질병급명학유인，병정3 d～2년。림상표현제기출질병표현외，균유불동정도적피부어반、기육종창동통、아간삼혈、혈뇨、혈편、복강혈종등출혈표현；실험실검사위중요적의거，표현위aPTT연장，위94．9±20．9초，혈장혼합시험aPTT불능피정상혈장규정，FⅧ：C강저위4．5±6．7％，FⅧ억제물적도재22．4±24．3 Bu；이상병례치료근거출혈정황급기출질병여전신상황，여지혈、소제항체조시，획득완전완해19례，부분완해5례，사망1례。기중5례사용리타석단항치료적환자균획득완전완해。획완해적24례，안FⅧ억제물적도수평분위3조，기여출혈적엄중정도、aPTT、FⅧ：C、획득부분완해적시간등인소진행분석，현시병무명학적상관성。상술병례경치료후림상증상호전，FⅧ활성승고지50％시간위23．8±13．3 d，FⅧ활성회복정상시간위86．2±76．8 d；FⅧ억제물적도감소50％적시간28±16．7 d，FⅧ억제물적도≤0．6 Bu혹전음시간156±230．5 d。결론이상병례경급조진단、급시보충응혈인자여응용면역억제약물후，출혈증상가명현감경，병정완해，단요FⅧ항체전음칙수교장시간치료급결합기타요법，리타석단항연합면역치료이취득교호료효，치득진일보연구。
Objective To discuss the clinical features ,diagnosis and treatment of acquired hemophilia A( AHA) .Methods To analyse the clinical data of 25 cases of AHA with clinical manifestations ,laboratory examination,diagnosis and curative effect.Results Of the 25 patients,13 were male,12 female;age 53.28 ± 19 .78 y;11 cases hadn ’ t basic disease and inducement before the onset of AHA ,the course of AHA was 3 d~2 years.In addition to clinical manifestations of basic disease ,The manifestations included different degrees of skin ecchymosis ,muscle swelling and pain ,gingival bleeding ,hematuria ,bloody stool ,abdominal hematoma . Abnormal laboratory examination indexes:aPTT extension,94.9 ±20.9 s;Plasma aPTT hybrid test cannot be corrected with normal plasma;FⅧ:C decreased,4.5 ±6.7%;FⅧinhibitor level was 22.4 ±24.3 Bu.After hemostasis and removal of blood coagulation factor antibody measures ,19 patients achieved complete remis-sion,partial remission in 5 cases,1 cases of death.5 patients achieved complete remission treated with ritux-imab .24 cases of remission were divided into 3 groups ,according to the FⅧinhibitor titers .There was no clear correlation between the titers of the FⅧinhibitor level with the severity of bleeding ,aPTT,FⅧ:C,the time of partial remission .The clinical symptoms of above cases improved after treatment , the time of FⅧ levels In-creased by 50%was 23.8 ±13.3 d,the recovery time of FⅧlevels was 86.2 ±76.8 d,the time of FⅧinhib-itor levels decreased by 50%was 28 ±16.7 d,the clearance time of FⅧinhibitor was 156 ±230.5 d.Con-clusion Patients with AHA may achieved remission by the early diagnosis ,timely supplement of blood coagu-lation factor and application of immunosuppressive drugs , and the bleeding symptoms can be significantly re-duced.But a long course of treatment and combination with other therapies are necessary to clear FⅧinhibi-tor.Rituximab combined with immunotherapy has achieved good curative effect ,and it is worth further study .