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: Yanhui Yang, Feng Xue, Hao Shi, Hongmei Wang, Lei Zhang, Linxiang Ji, Renchi Yang
• Journal: Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis (Clin Appl Thromb Hemost) Vol. 21 Issue 1 Pg. 35-40 (Jan 2015) ISSN: 1938-2723 [Electronic] United States
• PMID: 23677912 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: © The Author(s) 2013.

Chemical References

• Adrenal Cortex Hormones
• Antibodies, Monoclonal, Murine-Derived
• Autoantibodies
• Blood Coagulation Factors
• Immunosuppressive Agents
• prothrombin complex concentrates
• Rituximab
• Cyclophosphamide
• Factor VIII

Topics

• Adolescent
• Adrenal Cortex Hormones (administration & dosage)
• Adult
• Aged
• Antibodies, Monoclonal, Murine-Derived (administration & dosage)
• Autoantibodies (blood)
• Blood Coagulation Factors (administration & dosage)
• China
• Cyclophosphamide (administration & dosage)
• Factor VIII (antagonists & inhibitors, immunology)
• Female
• Hemophilia A (blood, immunology, therapy)
• Hemorrhage (therapy)
• Humans
• Immunosuppressive Agents (administration & dosage)
• Male
• Middle Aged
• Remission Induction (methods)
• Retrospective Studies
• Rituximab
• Young Adult