title: Acquired hemophilia a: retrospective analysis of 49 cases from a single Chinese hemophilia center.
abstract: Acquired hemophilia A (AHA) is a rare bleeding disorder caused by the autoantibody directed against factor VIII in patients without previous history of a bleeding disorder. We retrospectively analyzed the characteristics and outcomes of 49 patients with AHA diagnosed in our center from February 1994 to October 2012. Twenty-four patients with acute bleeding episodes were treated with prothrombin complex concentrate (PCC) at a relative low dose of 30 to -50 U/kg/d and achieved good outcomes without any adverse reaction. Corticosteroids alone or in combination with cyclophosphamide were used as the first-line therapy to eradicate the inhibitors. In 39 evaluable patients, 35 (89.7%) achieved complete remission (CR). This study demonstrates that when bypassing agents such as recombinant activated factor VII and activated PCCs are not affordable or available, low dose PCC is effective and safe to control acute bleeding in patients with AHA. First-line therapy achieved good outcomes with a CR rate of 89.7%.
authors.affiliation: Institute of Hematology and Hospital of Blood Diseases, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin, China.
authors.forename: Yanhui
authors.initials: Y
authors.lastname: Yang
authors.affiliation: Institute of Hematology and Hospital of Blood Diseases, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin, China.
authors.forename: Feng
authors.initials: F
authors.lastname: Xue
authors.affiliation: Institute of Hematology and Hospital of Blood Diseases, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin, China.
authors.forename: Hao
authors.initials: H
authors.lastname: Shi
authors.affiliation: Institute of Hematology and Hospital of Blood Diseases, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin, China.
authors.forename: Hongmei
authors.initials: H
authors.lastname: Wang
authors.affiliation: Institute of Hematology and Hospital of Blood Diseases, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin, China.
authors.forename: Lei
authors.initials: L
authors.lastname: Zhang
authors.affiliation: Institute of Hematology and Hospital of Blood Diseases, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin, China.
authors.forename: Linxiang
authors.initials: L
authors.lastname: Ji
authors.affiliation: Institute of Hematology and Hospital of Blood Diseases, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin, China rcyang65@163.com.
authors.forename: Renchi
authors.initials: R
authors.lastname: Yang
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000008:administration & dosage; D000328:Adult; D000368:Aged; D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage; D001323:Autoantibodies / Q000097:blood; D001779:Blood Coagulation Factors / Q000008:administration & dosage; D002681:China; D003520:Cyclophosphamide / Q000008:administration & dosage; D005169:Factor VIII / Q000037:antagonists & inhibitors / Q000276:immunology; D005260:Female; D006467:Hemophilia A / Q000097:blood / Q000276:immunology / Q000628:therapy*; D006470:Hemorrhage / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008297:Male; D008875:Middle Aged; D012074:Remission Induction / Q000379:methods; D012189:Retrospective Studies; D000069283:Rituximab; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D001779:Blood Coagulation Factors; D007166:Immunosuppressive Agents; C025667:prothrombin complex concentrates; D000069283:Rituximab; D003520:Cyclophosphamide; D005169:Factor VIII
keywords: PCC; acquired hemophilia A; immunosuppressive therapy; inhibitor