TAILIEUCHUNG - Chapter 110. Coagulation Disorders

Deficiencies of coagulation factors have been recognized for centuries. Patients with genetic deficiencies of plasma coagulation factors exhibit lifelong recurrent bleeding episodes into joints, muscles, and closed spaces, either spontaneously or following an injury. The most common inherited factor deficiencies are the hemophilias, X-linked diseases caused by deficiency of Factor (F) VIII (hemophilia A) or Factor IX (FIX, hemophilia B). | Chapter 110. Coagulation Disorders Deficiencies of coagulation factors have been recognized for centuries. Patients with genetic deficiencies of plasma coagulation factors exhibit lifelong recurrent bleeding episodes into joints muscles and closed spaces either spontaneously or following an injury. The most common inherited factor deficiencies are the hemophilias X-linked diseases caused by deficiency of Factor F VIII hemophilia A or Factor IX FIX hemophilia B . Rare congenital bleeding disorders due to deficiencies of other factors including FII prothrombin FV FVII FX FXI FXIII and fibrinogen are usually inherited in an autosomal recessive manner Table 110-1 . Advances in characterization of the molecular bases of clotting factor deficiencies have contributed to a better understanding of the disease phenotypes and may allow more targeted therapeutic approaches through the development of small molecules recombinant proteins or cell and gene-based therapies. Table 110-1 Genetic and Laboratory Characteristics of Inherited Coagulation Disorders Laboratory Abnormalit ya Clotting Inheritan Prevalen aPT PT TT Minimu Treatment Plas Factor Deficien cy ce ce in General Populati on T m Hemosta tic Levels ma HalfLife Fibrinoge n AR 1 in 1 000 00 0 100 mg dL Cryoprecipit ate 2 4 d Prothrom bin AR 1 in 2 000 00 0 - 20 30 FFP PCCs 3 4 d Factor V AR 1 in 1 000 00 0 - 15 20 FFP 36 h Factor VII AR 1 in 500 000 15 20 FFP PCCs 4 6 h Factor VIII X-linked 1 in 5 000 30 FVIII concentrate s 8 12 h Factor IX X-linked 1 in 30 000 30 FIX concentrate 18 24 h s Factor X AR 1 in 1 000 00 0 - - 15-20 - FFP PCCs 40-60 h Factor XI AR 1 in 1 000 00 0 - - 15-20 FFP 40-70 h Factor XII AR ND b -- b 60 h HK AR ND b -- b 150 h Prekallikr AR ein ND b -- b 35 h Factor AR XIII 1 in 2 000 00 0 - - 2-5 - Cryoprecipit 11-14 ate d aValues within normal range - or prolonged . bNo risk for bleeding treatment is not indicated. Abbreviations HK high-molecular weight kininogen AR autosomal recessive aPTT activated

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