A study of 65 patients with acquired hemophilia A in Taiwan
Journal
Journal of the Formosan Medical Association
Journal Volume
114
Journal Issue
4
Pages
321-327
Date Issued
2015
Author(s)
Abstract
Background/Purpose: Acquired hemophilia A (AHA) is a rare disorder that has not been comprehensively reported in the Chinese population. Treatment-related fatal sepsis (TRS), other than hemorrhage, is the leading cause of death in patients with AHA. However, researchers have not systematically evaluated salient parameters, to determine their association with the risk of TRS in this rare disorder. This study reports the salient features of AHA in Chinese patients and presents possible factors associated with TRS. Methods: Sixty-five Chinese patients with AHA, including 42 men and 23 women, were studied retrospectively. Results: The median age was 64 years (range=18-94 years). The features, laboratory findings, and outcomes of various therapies designed to arrest acute bleeding and eliminate autoantibodies against the factor VIII coagulant protein (VIIIi) were comparable to those previously reported. The complete response (CR) rate was 60%, and the median time to CR was 16 weeks. Ten patients (15%) died of bleeding related to FVIIIi by the end of the median follow-up period of 115 months. The estimated 1- and 5-year hemorrhage-related mortality rates were 15% and 22%, respectively. The absence of CR totherapy was the only independent factor associated with shorter survival. The rate ofTRS was 20%, and the use of a rituximab-based (Rb) regimen (odds ratio=8.0, 95% CI, 1.1-68.2) and platelet<1.5×1011/L at diagnosis (odds ratio=38.5, 95% CI, 1.3-1107.6) were the two significantly independent factors associated with TRS. Conclusion: The salient features of AHA and treatment outcomes of the patients in this study are similar to those of other patients. Two independent factors (the use of a Rb regimen and platelet<1.5×1011/L) were significantly associated with TRS. ? 2013.
SDGs
Other Subjects
activated prothrombin complex; autoantibody; blood clotting factor 8; cytotoxic agent; prednisolone; prothrombin complex; recombinant blood clotting factor 9; rituximab; autoantibody; immunosuppressive agent; rituximab; adult; aged; Article; bleeding; cell survival; drug fatality; drug induced disease; female; follow up; hemophilia A; human; major clinical study; male; medical record review; mortality; retrospective study; sepsis; survival; Taiwan; thrombocyte; treatment outcome; very elderly; adolescent; bleeding; blood; chemically induced; hemophilia A; middle aged; multivariate analysis; prognosis; register; remission; sepsis; statistical model; young adult; Adolescent; Adult; Aged; Aged, 80 and over; Autoantibodies; Female; Hemophilia A; Hemorrhage; Humans; Immunosuppressive Agents; Logistic Models; Male; Middle Aged; Multivariate Analysis; Prognosis; Registries; Remission Induction; Retrospective Studies; Rituximab; Sepsis; Taiwan; Treatment Outcome; Young Adult
Publisher
Elsevier
Type
journal article