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Hemophilia A is caused by a deficiency or absence of functional coagulation factor VIII
Hemophilia A occurs in 1 in 5,000 live male births.
Hemophilia A is 4x more common than Hemophilia B
70% of hemophilia patients inherit the faulty gene; however, 30% are spontaneous and occur in patients with no family history of the disease.
The primary symptom is uncontrolled, often spontaneous bleeding in different areas of the body
Hemophilia A. National Hemophilia Foundation. Accessed February 6, 2024. https://www.hemophilia.org/Bleeding-Disorders/Types-of-Bleeding-Disorders/Hemophilia-A
Data & Statistics on Hemophilia. CDC.gov. Updated September 14,2020. Accessed February 6, 2024. https://www.cdc.gov/ncbddd/hemophilia/data.html.
Hemophilia is a predominately male, X-linked genetic disorder.
Inherited or spontaneous mutations in the F8 gene result in a deficiency or absence of functional coagulation factor VIII (FVIII)
70% of hemophilia patients inherit the faulty gene; however, 30% are spontaneous and occur in patients with no family history of the disease.
The majority of carriers (XX) are asymptomatic.
Callaghan & Kaufman. Rec Adv Thromb Hemost 2008;45–67.
Mannucci et al. N Engl J Med 2001;344(23):1773–9.
Srivastava et al. Haemophilia 2013;19:e1–47.
Joint bleeds account for 70-80% of bleeds in patients with hemophilia.
As little as one joint bleed can lead to irreversible joint damage.
Prophylaxis initiated prior to the onset of arthropathy has been shown to preserve joint function in adulthood.
High trough levels are important to prevent bleeds and protect joints; in patients with hemophilia A, those with low factor levels (<5%) have the highest risk for joint bleeds, while those with factor levels of ≥15% had a very low risk approximating no expected joint bleeds.
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Rodriguez-Merchan. Adv Prev Med. 2012;2012:201-271; Clausen, et al. Haemophilia 2014;20(6):747–755; Den uijl, et al. Haemophilia. 2011;17(6):849-853.
FVIII concentrates are the treatment of choice.
Each unit of FVIII/kg infused will raise the plasma FVIII level approximately 2 IU/dL.
Desmopressin (DDAVP) may be utilized in patients with mild or moderate disease.
Individual response should be tested prior to therapeutic use (significant differences between individuals).
Srivastava, et al. Hemophilia 2013;19:e1–e47.
Treatment Guidelines
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