44例获得性血友病A的病因、临床表现及诊疗的探讨

The Etiology,Clinical Manifestation,Diagnosis and Treatment Effect in Patients with Acquired Hemophilia-A

目的探讨获得性血友病A(AH-A)的病因、临床表现、诊断与治疗效果,为临床对AH-A疾病诊断、治疗及预后并发症的评估提供依据。方法本院选择于2014年1月至2016年12月接受治疗的AH-A患者44例作为观察对象,对其病因、临床表现、诊治及实验室检查等信息进行统计分析。结果研究发现40.91%的AH-A患者无明确病因,其余引发AH-A的基础病因主要包括风湿性疾病、产后并发症及术后大出血等,所占比例依次为15.91%、9.09%及9.09%。AH-A疾病的临床表征主要包括皮肤瘀斑、软组织血肿、肌肉血肿、失血性贫血及腹腔血肿等,所占比例依次为93.18%、72.73%、52.27%、25.0%及20.45%。治疗后的a PTT与FⅧ:Ab显著低于治疗前,差异均有统计学意义(t=4.842,P=0.016;t=5.427,P=0.004);治疗后FⅧ:C的水平显著高于治疗前,差异均有统计学意义(t=4.881,P=0.028)。Ⅷ抑制物的水平高低与FⅧ:C及a PTT长短、出血严重性无直线相关性,差异均无统计学意义(P>0.05)。结论结合患者的病症、年龄、并发症、a PTT、FⅧ:C及FⅧ:Ab等指标综合评估之后制定科学有效的治疗方案,可以大幅度地降低AH-A的病情恶化,降低AH-A病死率,对于AH-A疾病的临床管理和防治具有十分重要的指导意义。

Objective To explore the etiology, clinical manifestation, diagnosis and treatment effect in patients with acquired hemophilia A （AH-A）in hospital, providing the basis for the diagnosis, treatment and prognosis of complications in AH-A disease. Methods A total of 44 cases with AH-A who were treated in the hospital from Jan. 2014 to Dec. 2016 were recruited as the study objects. The information of the etiology, clinical manifestation, diagnosis and treatment and laboratory examination were observed by Statistical analysis. Results The study found that the main etiology and complications of the AH-A disease included rheumatoid disease, postpartum complications and postoperative bleeding, etc. , the proportion were 15.91%, 9.09% and 9. 09% in turn. The clinical manifestations of the AH-A disease included skin ecchymosis, hematoma of soft tissue, muscle hematoma, hemorrhagic anemia and intraperitoneal hematoma, etc. , the proportion were 93.18% ,72. 73% ,52.27% ,25.0% and 20. 45% in turn. The aPTT and F VIII： Ab were significantly lower after treatment than that before treatment, and the difference was statistically significant（ t = 4. 842, P = 0. 016 ; t = 5.427, P = 0. 004）. The level of FVIII ： C was significantly higher after treatment than that before treatment,and the difference was statistically significant （t = 4. 881, P = 4. 881 ）. The levels of V-I inhibitor was no linear correlation with F VIII： C, the length of aPTT, and the hemorrhage severity, and the difference were statistically significant （P 〉 0. 05 ）. Conclusion The scientific and effective treatment combined with the patient＇ s symptoms, age, complications, aPTT, F VIII ： C and F VIII ： Ab measures, it could reduce greatly the deterioration of AH- A, reducing the AH- A mortality, and having important guiding significance on the clinical management and prevention and control for the AH-A disease.