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Haemophilia A affects about 1 in 5,000–10,000, while haemophilia B affects about 1 in 40,000 males at birth. [2] [5] As haemophilia A and B are both X-linked recessive disorders, females are rarely severely affected. [8] Some females with a nonfunctional gene on one of the X chromosomes may be mildly symptomatic. [8]
Joint capsule. Haemophilia A's phenotype has a quite wide range of symptoms encompassing both internal and external bleeding episodes. Individuals with more severe haemophilia tend to experience more intense and frequent bleeding, whereas those with mild haemophilia typically exhibit milder symptoms unless subjected to surgical procedures or significant trauma.
Factor VIII is produced in the liver's sinusoidal cells and endothelial cells outside the liver throughout the body. This protein circulates in the bloodstream in an inactive form, bound to another molecule called von Willebrand factor , until an injury that damages blood vessels occurs. [ 8 ]
Coagulation factor concentrates are used to treat hemophilia, to reverse the effects of anticoagulants, and to treat bleeding in people with impaired coagulation factor synthesis or increased consumption. Prothrombin complex concentrate, cryoprecipitate and fresh frozen plasma are commonly used coagulation factor products.
Acquired haemophilia A (AHA) is a rare but potentially life-threatening bleeding disorder characterized by autoantibodies directed against coagulation factor VIII.These autoantibodies constitute the most common spontaneous inhibitor to any coagulation factor and may induce spontaneous bleeding in patients with no previous history of a bleeding disorder.
This is an incomplete list, which may never be able to satisfy certain standards for completion.. There are many conditions of or affecting the human hematologic system—the biological system that includes plasma, platelets, leukocytes, and erythrocytes, the major components of blood and the bone marrow.
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In hemophilia it may occur spontaneously, and recurrent hemarthroses are a major cause of disability in that patient group due to hemophilic arthropathy, requiring synovectomy, joint replacement [9] and increased medical therapy to prevent further bleeding episodes.
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