title: A study of 65 patients with acquired hemophilia A in Taiwan.
abstract: OBJECTIVE
  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 to therapy was the only independent factor associated with shorter survival. The rate of TRS 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 × 10(11)/L at diagnosis (odds ratio = 38.5, 95% CI, 1.3-1107.6) were the two significantly independent factors associated with TRS.
  
  
  CONCLUSIONS
  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 × 10(11)/L) were significantly associated with TRS.
authors.affiliation: Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan.
authors.forename: Shang-Yi
authors.initials: SY
authors.lastname: Huang
authors.affiliation: Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan; Haemophilia Center, National Taiwan University Hospital, Taipei, Taiwan.
authors.forename: Woei
authors.initials: W
authors.lastname: Tsay
authors.affiliation: Department of Internal Medicine, Chang-Hua Christian Hospital, Chang-Hua, Taiwan.
authors.forename: Shyuann-Yuh
authors.initials: SY
authors.lastname: Lin
authors.affiliation: Department of Laboratory Medicine, National Taiwan University Hospital, Taipei, Taiwan; Haemophilia Center, National Taiwan University Hospital, Taipei, Taiwan.
authors.forename: Szu-Chun
authors.initials: SC
authors.lastname: Hsu
authors.affiliation: Department of Laboratory Medicine, National Taiwan University Hospital, Taipei, Taiwan.
authors.forename: Mei-Hwa
authors.initials: MH
authors.lastname: Hung
authors.affiliation: Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan; Department of Laboratory Medicine, National Taiwan University Hospital, Taipei, Taiwan; Haemophilia Center, National Taiwan University Hospital, Taipei, Taiwan; Department of Internal Medicine, Chang-Hua Christian Hospital, Chang-Hua, Taiwan. Electronic address: 111710@cch.org.tw.
authors.forename: Ming-Ching
authors.initials: MC
authors.lastname: Shen
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood; D005260:Female; D006467:Hemophilia A / Q000188:drug therapy*; D006470:Hemorrhage / Q000401:mortality; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage* / Q000009:adverse effects; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011379:Prognosis; D012042:Registries; D012074:Remission Induction; D012189:Retrospective Studies; D000069283:Rituximab / Q000008:administration & dosage* / Q000009:adverse effects; D018805:Sepsis / Q000139:chemically induced*; D013624:Taiwan; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D007166:Immunosuppressive Agents; D000069283:Rituximab
keywords: Chinese, Taiwan; acquired hemophilia A; platelet, complete remission; progonsis; rituximab; sepsis