FINALS

• Hemorrhagic disorders


  Disorders characterized by excessive or uncontrolled bleeding
• Acquired coagulopathies covered in this module


  • ACOTS
  • Liver disease coagulopathy
  • Chronic renal failure
  • Vitamin K deficiency
  • Auto-anti VIII inhibitor and acquired hemophilia
  • Acquired vWD
  • DIC
• Congenital coagulopathies covered in this module


  • vWD
  • Hemophilia A
  • Hemophilia B
  • Hemophilia C
  • Other single-factor deficiencies
• Localized bleeding


  Bleeding in a single location, indicating injury, infection, tumor, or an isolated vessel defect
• Generalized bleeding


  Bleeding at multiple sites, spontaneous and recurring, requiring physical intervention and transfusion, potential evidence of hemostatic disorders
• Mucocutaneous hemorrhage


  Bleeding from mucous membranes and skin, associated with primary hemostasis defects like vascular problems and platelet defects
• Anatomic hemorrhage


  Internal bleeding into joints, muscles, CNS, often seen in congenital defects of secondary hemostasis or coagulopathies
• Acquired hemorrhage


  Associated with a disease, not with relatives, diagnosed by patient history
• Congenital hemorrhage


  Uncommon, usually diagnosed in infancy or early childhood, confirmed by family history
• Quantitative coagulation factor abnormality


  Defect in the synthesis of the factor, resulting in decreased concentration
• Qualitative coagulation factor abnormality


  Synthesis of a defective factor, with normal amounts of inactive or abnormally functioning factors
• ACOTS (Acute-Coagulopathy of Trauma-Shock)


  Triggered by injury-related acute inflammation, platelet activation, tissue factor release, hypothermia, acidosis, and hypoperfusion, accounts for most fatal hemorrhages

  
• Liver disease coagulopathy


  Multiple factor deficiencies, abnormalities of fibrinolysis, thrombocytopenia and qualitative platelet abnormalities, factor VII deficiency most pronounced

  
• Chronic renal failure


  Associated with platelet dysfunction, mild to moderate bleeding
• DIC (Disseminated intravascular coagulation)


  Generalized over-activation of the coagulation and fibrinolytic systems in the circulating blood, coagulation and fibrinolysis occur simultaneously
• Vitamin K deficiency


  Causes impaired synthesis of factors II, VII, IX and X, leading to bleeding
• Factor inhibitors


  Antibodies produced by the body that inhibit coagulation, including autoanti-VIII, lupus inhibitor, and inhibitors to other factors
• Acquired vWF deficiency


  Decreased production, presence of autoantibody, or adsorption to abnormal cell surfaces, leading to mucocutaneous bleeding
• von Willebrand's Disease (vWD)


  Most common hereditary hemorrhagic disorder, caused by absent or abnormal von Willebrand factor, leading to impaired platelet adhesion and factor VIII clearance
• Types of congenital vWD

  • Type 1 (quantitative deficiency)
  • Type 2 (qualitative abnormalities with 4 subtypes)
  • Type 3 (absent or nearly absent vWF)
• Fibrinogen deficiencies


  Afibrinogenemia (no measurable fibrinogen), hypofibrinogenemia (low fibrinogen), and dysfibrinogenemia (functionally abnormal fibrinogen), leading to spontaneous bleeding
• Factor II (prothrombin) deficiency


  Rarest of the factor deficiencies, with hypoprothrombinemia (reduced prothrombin activity and antigen) and dysprothrombinemia (reduced function)
• Afibrinogenemia


  No measurable fibrinogen; severe bleeding
• Hypofibrinogenemia


  ‹100 mg/dL fibrinogen; heterozygous expression
• Dysfibrinogenemia


  Functionally abnormal fibrinogen; Substitution of amino acids in fibrinogen's polypeptide chain
• Afibrinogenemia


  • RECESSIVE
• Hypofibrinogenemia and Dysfibronogenemia


  • DOMINANT
• Factor I Deficiency


  Spontaneous bleeding of mucosa, intestines, and intracranial sites; Normal platelet count; Prolonged bleeding time, PT, aPTT and thrombin time; Blood will not clot in Hereditary afibrinogenemia; PT/aPTT not corrected with fresh/aged serum
• Cryoprecipitate


  Treatment of choice in the US for Factor I Deficiency
• FFP


  May be given for Factor I Deficiency; fibrinogen concentrate is now available
• Factor II Deficiency


  Rarest of the factor deficiencies; Autosomal recessive; Hypoprothrombinemia - Deficiency, Dysprothrombinemia - Dysfunction
• Factor II Deficiency - Clinical


  Mild bleeding symptoms
• Factor II Deficiency - Treatment


  Prothrombin complex concentrates are the treatment of choice
• Factor II Deficiency - Acquired


  Commonly found in association with vitamin K deficiency and Coumadin therapy
• Factor II Deficiency - Laboratory


  PT and aPTT are abnormal; Stypven time abnormal; PT/aPTT not corrected by adsorbed plasma and aged serum
• Factor V Deficiency


  Congenital deficiency has been designated parahemophilia; Autosomal recessive; Bleeding may be mucosal up to severe; Heterozygous individuals are usually asymptomatic
• Factor V Deficiency - Treatment


  The only suitable replacement product available is FFP; No available factor V concentrates
• Factor V Deficiency - Laboratory


  PT and aPTT abnormal; Stypven time abnormal; Corrected with adsorbed plasma
• Factor VII Deficiency


  Also known as Alexander's disease; Rare, autosomal recessive; Therapeutic options include fresh frozen plasma, prothrombin complex concentrates, and recombinant FVIIa
• Factor VII Deficiency - Clinical


  Soft tissue bleeding