Quick topics

Acquired haemophilia A is a rare but potentially life-threatening cause of bleeding1,2

Acquired haemophilia A is caused by autoantibodies to factor VIII, which result in sudden bleeds in patients with no history of bleeding disorders

Bleeding is usually severe and without treatment can lead to death, with the overall mortality rate in acquired haemophilia A ranging between 9% and 42%2-4

autoantibodies illustration

Acquired haemophilia A is distinct from congenital haemophilia2

Acquired haemophilia A Congenital haemophilia A
Condition Acute onset of severe or life-threatening bleeding or widespread subcutaneous bleeding2 Chronic9
Cause Autoantibodies to factor VIII2,6 Inherited, congenital deficiency in factor VIII2,6
Patients Occurs mostly later in life; gender occurrence is about equal6-8 Particularly affects males6
Bleed location Usually into the skin, muscles or soft tissue and mucous membranes, and not into joints2,6 Primarily joints, also muscle and soft tissue2,6

Spot the signs

Typical signs of acquired haemophilia A include:


ONE

Large areas of subcutaneous haemorrhage2

TWO

Bleeding into the muscles vs into the joints10

THREE

Gastrointestinal, genitourinary and retroperitoneal bleeding10

FOUR

Extensive bleeding after invasive procedures2

80% of patients present with widespread subcutaneous bleeds2,8

Sites of bleeding in patients with acquired haemophilia A in a 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors’ Organisation (n=172)8


Chart of % of cohort with each bleeding subtype

Adapted from Collins et al 2007.
*Many patients had more than one type of bleeding.

Example acquired haemophilia A presentations


Diffused abdominal skin bleeding in right upper extremity
Diffused right upper extremity skin bleeding in a patient with acquired hemophilia A

Images from ‘Acquired hemophilia: a report of 4 cases’ by Karimi M, et al. This paper was published by Austin Publishing Group under a Creative Commons Attribution (CC BY) license.

SUMMARY

Identifying acquired haemophilia A

Unexplained bleeding with no history of bleeding disorders?2,5

Unexplained bruising could be a sign of acquired haemophilia A, a rare but potentially life-threatening condition1,2,5

Usually presents as widespread subcutaneous bleeding1,2