Chapter 110. Coagulation Disorders (Part 1)

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Harrison's Internal Medicine Chapter 110. Coagulation Disorders Coagulation Disorders: Introduction 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)....

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Chapter 110. Coagulation Disorders (Part 1) Harrison's Internal Medicine > Chapter 110. Coagulation Disorders Coagulation Disorders: Introduction 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 Laborator y Abnormalitya Clo In Pr a M Trea P tting heritance evalence PTT T T inimum tment lasma Factor in Hemosta Half- Deficienc General tic Life y Populati Levels on Fib AR 1 + 10 Cryo 2 rinogen in 0 mg/dL precipitate –4 d 1,000,000 Pro AR 1 + 20 FFP/ 3
thrombin in –30% PCCs –4 d 2,000,000 Fac AR 1 + 15 FFP 3 tor V in /– /– –20% 6h 1,000,000 Fac AR 1 – 15 FFP/ 4 tor VII in –20% PCCs –6 h 500,000 Fac X- 1 + 30 FVII 8 tor VIII linked in 5,000 % I –12 h concentrate s Fac X- 1 + 30 FIX 1 tor IX linked in 30,000 % concentrate 8–24 h s Fac AR 1 + 15 FFP/ 4
tor X in /– /– –20% PCCs 0–60 h 1,000,000 Fac AR 1 + 15 FFP 4 tor XI in –20% 0–70 h 1,000,000 b b Fac AR N + 6 tor XII D 0h b b HK AR N + 1 D 50 h b b Pre AR N + 3 kallikrein D 5h Fac AR 1 – 2– Cryo 1 tor XIII in /– 5% precipitate 1–14 d 2,000,000
a Values within normal range (–) or prolonged (+). b No risk for bleeding, treatment is not indicated. Abbreviations: HK, high-molecular weight kininogen; AR, autosomal recessive; aPTT, activated partial thromboplastin time; PT, prothrombin time; TT, thrombin time; ND, not determined; FFP, fresh frozen plasma; PCCs, prothrombin complex concentrates.