Acquired haemophilia: Epidemiology, clinical presentation, diagnosis and treatment

Med Clin (Barc). 2017 Apr 7;148(7):314-322. doi: 10.1016/j.medcli.2016.11.030. Epub 2017 Jan 22.
[Article in English, Spanish]


The development of circulating autoantibodies able to inhibit some coagulation proteins induces severe or even life-threatening bleeding. This disorder is called acquired haemophilia. This is a rare disease, although its impact may be underestimated because of the lack of records, the lack of knowledge by many specialists, the complexity of the laboratory diagnosis and, finally, because of the fulminant clinical presentation that often precludes diagnosis. Several studies established that mortality ranges between 9 and 33%. Not only haematologists but all physicians should be trained to follow the right steps to diagnose these patients as soon as possible in order to reduce such mortality rates. This review approaches the basic concepts dealing with the diagnosis and management of these patients and intends to assist physicians in identifying patients under suspicion of acquired haemophilia to correctly manage them and refer them to the appropriate Haemostasis Unit.

Keywords: Acquired haemophilia; Anti-Factor VIII autoantibodies; Antihaemorragic treatment; Autoanticuerpos antifactor viii; Eradication of factor VIII Inhibitor; Erradicación del inhibidor del factor viii; Factor VIII; Factor VIII Inhibitor; Hemofilia adquirida; Inhibidor del factor viii; Tratamiento antihemorrágico.

Publication types

  • Review

MeSH terms

  • Biomarkers / blood
  • Drug Therapy, Combination
  • Hematologic Agents / therapeutic use
  • Hemophilia A* / blood
  • Hemophilia A* / diagnosis
  • Hemophilia A* / epidemiology
  • Hemophilia A* / therapy
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Plasmapheresis
  • Prognosis


  • Biomarkers
  • Hematologic Agents
  • Immunosuppressive Agents

Supplementary concepts

  • Factor 8 deficiency, acquired