Inherited Coagulation Disorders Associated with Bleeding

Chapter: Inherited Coagulation Disorders Associated with Bleeding
McMaster Section Editor(s): Mark Crowther
Section Editor(s) in Interna Szczeklika: Andrzej Hellmann, Bogdan Ochrem
McMaster Author(s): Alfonso Iorio, Mark Crowther
Author(s) in Interna Szczeklika: Jerzy Windyga
Additional Information


Inherited coagulation disorders are most frequently caused by a deficiency or dysfunction of one of the plasma coagulation factors (hemophilia A [factor VIII] or B [factor IX]). Inherited deficiencies of factor XII, prekallikrein, and high-molecular-weight kininogen cause prolongation of the activated partial thromboplastin time but are asymptomatic and require no treatment. Rare deficiencies of fibrinogen, prothrombin, and factors V, VII, X, XI, or XIII cause bleeding of varied severity. Treatment is based on replacement of the deficient coagulation factors.

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