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Haemophilia (British English), or hemophilia (American English) [6] (from Ancient Greek αἷμα (haîma) 'blood' and φιλία (philía) 'love of'), [7] is a mostly inherited genetic disorder that impairs the body's ability to make blood clots, a process needed to stop bleeding.
Haemophilia B, also spelled hemophilia B, is a blood clotting disorder causing easy bruising and bleeding due to an inherited mutation of the gene for factor IX, and resulting in a deficiency of factor IX. It is less common than factor VIII deficiency (haemophilia A). [3]
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.
A bruise, also known as a contusion, is a type of hematoma of tissue, [3] the most common cause being capillaries damaged by trauma, causing localized bleeding that extravasates into the surrounding interstitial tissues. Most bruises occur close enough to the epidermis such that the bleeding causes a visible discoloration.
Idiopathic thrombocytopenic purpura (ITP) is the condition of having a low platelet count (thrombocytopenia) of no known cause . Recurrent thrombosis: Hemophilia: Hemophilia A: Hemophilia B: Hemophilia C: Von Willebrand disease: Antiphospholipid syndrome: Thrombocytopenia: Glanzmann's thrombasthenia: Wiskott–Aldrich syndrome
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.
The slow process of reabsorption of hematomas can allow the broken down blood cells and hemoglobin pigment to move in the connective tissue. For example, a patient who injures the base of their thumb might cause a hematoma, which will slowly move all through their finger within a week. Gravity is the main determinant of this process.
[4] [10] The prolongation of the activated partial thromboplastin time should completely correct with a 1:1 mixing study with normal plasma if haemophilia C is present; in contrast, if a lupus anticoagulant is present as the cause of a prolonged aPTT, the aPTT will not correct with a 1:1 mixing study. [citation needed]
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