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Disorders of Plasma Clotting Factors

Disorders of Plasma Clotting Factors. Kaur, 2006. Fibrinogen. Prothrombin –Factor 2. Clotting Factors Defects. Decreased Synthesis Production of abnormal molecules Loss of consumption of Coagulation factors Inactivation of factors by inhitors or antibodies.

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Disorders of Plasma Clotting Factors

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  1. Disorders of Plasma Clotting Factors Kaur, 2006

  2. Fibrinogen

  3. Prothrombin –Factor 2

  4. Clotting Factors Defects • Decreased Synthesis • Production of abnormal molecules • Loss of consumption of Coagulation factors • Inactivation of factors by inhitors or antibodies

  5. Transmission of Clotting Factors Disorders Inherited • Sex linked Acquired • Vitamin K deficiency • Liver disease • Hemorrhage • DIC

  6. FACTOR DEFICIENCIES Table 25-1, p496

  7. Von Willebrand’s Disease • Autosomal • Consistently prolonged bleeding time • Mucocutaneous bleeding

  8. Production • Megakaryocytes and endothelial cells • Found in plasma • Stored in plasma granules and endothelial cells • Combines with VIII as VIII:vW • Allows VIII:C to concentrate at site of injury

  9. Correction • Normal plasma

  10. Signs and Symptoms • Mucocutaneous bleeding • Epistaxis • Ecchymosis • Easy bruising • GI bleeding • Menorrhagia • hemorrhage

  11. Function • Carrier protein for VIII:C • Inability of the platelet to adhere to the subendothelial surface

  12. LABORATORY ASSAYS • PT – Normal • APTT – Prolonged -corrects with pooled plasma • Platelets count – normal • Bleeding time – abnormal • Factor VIII:C activity • vWF-Ag concentration • Ristocetin aggregation

  13. TREATMENT • CONCENTRATES OF fviii • CRYOPRECIPITATE • vWF • DESMOPRESSIN

  14. HEMOPHILIA A • Most common hereditary coagulation disorder • Sex linked • Absence of VIII:C (procoagulant factor) • vWF:Ag is normal

  15. INHERITANCE • X-linked (only one chromosome carries the gene for active VIII) • Males inherit from mothers • Daughters carriers • Mutation or silent carriers

  16. Signs and Symptoms • Less than 1% have severe hemorrhage • Hemathrosis

  17. Characteristics of Factor • Acute phase reactant • Minor portion is linked as VIII:C • Major portion is VIII:vW

  18. Laboratory Findings • APTT – prolonged • PT – normal • Bleeding time – normal • Mixing studies with pooled plasma or adsorbed plasma corrects • APTT - Functional activity

  19. TREATMENT • Concentrates of Human Plasma • Purified heat treated lyophilized FVIII (most accepted) • Recombinant-DNA FVIII

  20. FACTOR IX – Christmas Factor/Plasma Thromboplastin Component • Synthesized in the liver • Vitamin k dependent • Intrinsic pathway activated by factor Xia and calcium ions • Activated by Russell viper venom

  21. AGED SERUM Aged serum is prepared by incubating normal serum for 24 hours at 37°C. Lacks • fibrinogen (I), • prothrombin (II), • Factor V, • Factor VIII. Present • Factors VII, • IX, • X, • XI, • XII

  22. ADSORBED PLASMA Platelet-poor plasma that is adsorbed with either barium sulfate or aluminum hydroxide Lacks the coagulation factors • factors II, VII, IX, X (the prothrombin group). Present • Factors V, VIII, XI, XII, and fibrinogen (I)

  23. Hemophilia B (Christmas Disease) • Factor IX deficiency • Sex linked • Acquired • Liver disease • Vitamin K deficiency • Oral anticoagulant therapy

  24. Serious complication • Development of antibodies (differs from Hemophillia A)

  25. Laboratory Findings • PT – Normal • Thrombin Time – Normal • Bleeding Time – Normal • APTT – prolonged • Correction of APTT with pooled normal plasma or aged serum

  26. Factor X

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