The Clinical Analysis Of 15 Patients Of Acquired Hemophilia A

Objective:To improve the recognition of the pathogenesis,clinical symptoms,laboratory findings and therapies of acquired hemophilia A(AHA).Methods:To retrospectively analyze the clinical charactstics,diagnosis,and therapies of 15 patients with AHA,who were diagnosed and treated in The Second Hospital of Hebei Medical University from 2014 to 2018.Results:Among the 15 patients,3 cases were male and 12 cases were female and the ratio of male to female was 1:4.The onset age was 24-87 years old,and the median age was 41 years old.Mucocutaneous hemorrhage and muscular hemorrhage were the main clinical features.Among the 15 patients,6(40%)had related causes,including 3 cases of postpartum,2 cases of autoimmune diseases,1 case of malignant tumor related to the blood system,and 9 cases(60%)were idiopathic.All patients had normal PT and prolonged APTT,with APTT values between 59.8-119.5s and a median of 93.6s.The APTT correction test showed that prolonged APTT could not be corrected by normal mixed plasma.Coagulation factor VIII(FVIII)activity(FVIII:C)was moderately and severely decreased in all patients,ranging from 0.2% to 3.2%,and FVIII inhibitors were detected in all patients,with the inhibitory titers between 4.4-131.2 BU and 93%(14/15)of high-titer inhibitors(>5 BU).Acute bleeding events were controlled in 14 patients after the application of prothrombin complex concentration(PCC)and fresh plasma,and the average PCC consumption was 43671 unit.Fourteen patients were treated with corticosteroid combined with cyclophosphamide,of which 5 patients were also treated with rituximab.The clearance time of inhibitors to F Ⅷafter immunosuppressive therapy was 21-72 days,with an average of 31.6 days.Conclusions:1.Acquire hemophilia A is a rare bleeding disease which can occur in both male and female at any age;2.The main manifestation is spontaneous bleeding,especially in skin,mucosa and muscle.Laboratory examination indicated the presence of prolonged APTT,decreased FVIII:C,and the detection of FVIII inhibitor,but the titer of FVIII inhibitor was not significantly correlated with the severity of bleeding.3.Bypass replacement agents are recommended as optimal antihemorrhagic treatment.Corticosteroid combined with cyclophosphamide is still considered as the main therapy to eradicate the inhibitors,and rituximab is suitable for second-line therapy for patients whose does not respond to corticosteroid or cyclophosphamide.