ACQUIRED HEMOPHILIA A

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Acquired hemophilia A: Low prevalence. High mortality.6-8

Acquired hemophilia A is a rare bleeding condition that is caused by the development of an autoantibody (inhibitor) to plasma coagulation factors, usually to factor VIII (FVIII).6,9 These autoantibodies inhibit coagulation by neutralizing FVIII activity function and accelerating its clearance from blood plasma.8 Patients with acquired hemophilia A present with bleeds that are often spontaneous.6 These bleeds are rarely intra-articular, and bleeds commonly are found in the skin, muscles, or soft tissues and mucous membranes.9

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9%-22%

Mortality associated with acquired hemophilia A has been estimated between 9% and 22%6

1.5 CASES PER MILLION/YEAR WORLDWIDE6

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~ 500 cases per year in the United States,
based on 2014 US Census Bureau Data6,7

SIMILAR INCIDENCE BETWEEN SEXES*10

Male

53.1%

men

Female

46.9%

women

*Based on the data from the EACH2 registry.

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The rarity of acquired hemophilia A and the corresponding inexperience of initial-contact physicians (typically ER physicians, geriatricians, obstetricians, rheumatologists, oncologists, and other non-hematologists) may contribute to delays in diagnosis.6 Patients with acquired hemophilia A usually do not have a personal or family history of bleeding episodes, as seen with congenital, non-acquired hemophilia A, and signs and symptoms of acquired hemophilia A differ.9 (See table below.)

This, in turn, often leads to postponed or ineffective treatment.6

Initial-contact physicians are advised to immediately consult with a hemophilia center or a hematologist with expertise in managing patients with inhibitors when a patient presents with abnormal bleeding.6

Acquired hemophilia A differs from congenital hemophilia A

Acquired vs Congenital Hemophilia A6,8,10,11
Characteristic Acquired Congenital
Genetic inheritance No known pattern Inherited
Incidence between sexes ~Equal, with an increased incidence in women of child-bearing age Primarily affects males
Patient population Mostly elderly, some postpartum females Identified early in life
FVIII relationship to bleeding severity Cannot be predicted by FVIII levels Correlation between FVIII levels and severity
Bleed location Mostly in skin, muscles, soft tissues, and mucous membranes Mostly in joints and also in muscles and soft tissues
Treatment administration In hospital or other specialized facility At-home self-administration
Treatment goals Immediate control of acute bleeding, long-term management of underlying causes Minimize bleeding episodes for lifelong disease management