Coagulation Disorders

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julieaburch
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85197
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Coagulation Disorders
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2011-05-11 11:17:26
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Coagulation Disorders
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Coagulation Disorders
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  1. What disease accounts for 99+% of all inherited bleeding disorders?
    Hemophilia
  2. Secondary Hemostasis is linked to what type of bleeding?
    Muscle & Joint Bleeding
  3. Will the PT be elevated, normal, or decreased when assessing a hemophiliac?
    Normal
  4. Will the PTT be elevated, normal, or decreased when assessing a hemophiliac?
    Elevated
  5. Coagulation Factor Ranges vary; however, most are ____ - _____%
    50-150
  6. Fibrinogen is expressed as mg/dl. What is its reference range?
    150-400 mg/dl
  7. A Factore VIII deficiency is linked to what disease? How common is this disease type in reference to the disease?
    • Hemophilia A
    • 80%
  8. A Factor IX deficiency is linked to what disease? How common is this disease type in reference to the disease?
    • Hemophilia B (Christmas Disease)
    • 20%
  9. A Factor XI deficiency is linked to what disease? How common is this disease type in reference to the disease?
    • Hemophilia C
    • <1%
  10. Hemophilia is said to be severe if its level is what? These patients experience many bleeding episodes, usually spontaneous. They rarely reach adulthood without treatment, and have no detectable factor.
    <1%
  11. Hemophilia is said to be moderate if its levels is what? These patients have occasional bleeding problems. They can reach adulthood without crippling effects, and their bleeding is often trauma induced
    1-5%
  12. Hemophilia is said to be mild if its level is what? These patients have rare bleeding episodes, and lead fairly active lives. Their hemophilia may not be discovered until adulthood, and they only really have problems at surgery or major trauma.
    6-30%
  13. As far as the PTT is concerned, Hemophilia is classified as severe if it is ____ - ______ secs (RR 25-36 sec).
    72-125
  14. As far as the PTT is concerned, Hemophilia is classified as moderate if it is ____ - ______ secs (RR 25-36 sec).
    45-72
  15. As far as the PTT is concerned, Hemophilia is classified as mild if it is ____ - ______ secs (RR 25-36 sec).
    33-45
  16. What population has a high incidence of Hemophilia C, even more so than the incidence of A & B within that population?
    Ashkenazi Jews
  17. What is the mode of inheritance for Hemophilia A & B?
    X-Linked
  18. This is an antibody against coagulation factors. It is measured in Bethesda units.
    Coagulation Inhibitor
  19. In the formation of this, repeated infusions of the missing factor are given, but the body recognizes the factor as "foreign" and therefore produces antibodies against it. What does this describe?
    Inhibitor Formation
  20. How is Hemophilia A treated? What are the contraindications?
    • Factor VIII Concentrate: recombinant or plasma derived
    • DDAVP: oral drug, for milder forms
    • Prophylactic: if severe
    • Contraindicated: Cryoprecipitate & FFP
  21. How is Hemophilia B treated? What are the contraindications?
    • Factor IX Concentrate: recombinant or plasma derived
    • Prophylactic: if severe
    • Contraindicated: FFP
  22. How is Hemophilia C treated?
    Fresh Frozen Plasma
  23. What Factor is von Willebrand Factor a carrier of? Loss of vWF results in a reduction in the half-life of this factor.
    Factor VIII
  24. What are the common clinical signs of von Willebrand's Disease?
    • Nose Bleeds
    • Easy Bruising and Petechia
    • Menorrhagia
    • Gum Bleeding
  25. Why do von Willebrand Disease patients not usually bleed in stress situations?
    Stress causes an increase in vWF
  26. What blood type has lower levels of vWF than the others?
    O
  27. What is the mode of inheritance for von Willebrand's Disease (usually)?
    Autosomal Dominant
  28. How can Types 1, 2, & 3 of von Willebrand's Disease be distinguished from one another?
    • Type 1: activity and antigen levels decreased, but not 0. Multimers present. Decreased levels.
    • Type 2: have factors, so no decreased levels; factors just don’t work
    • Type 3: no factors
  29. For milder forms of vWD, such as Type 1, what is the treatment? What is the long term treatment option? What are the contraindications?
    • DDAVP
    • Concentrate, usually plasma derived
    • Contraindications: Cryoprecipitate & FFP
  30. In this acquired disorder of coagulation, there is decreased synthesis of all factors except FVIII & vWF. Platelet dysfunction is seen, and the factors decrease as the disease gets more severe. This causes bleeding problems. A prolonged PT & PTT is seen, along with other hemostatic complications.
    Coagulation in Liver Disease
  31. Specific coagulation inhibitors are seen, in which antibodies are developed against specific factors. What are the most common factors that this occurs in?
    Factors VIII, IX, V, & XI
  32. This acquired coagulation defect is an autoimmune disease against the protein-phopholipid complex. It is a severe disease, and causes thrombosis in about 30% of patients. A prolonged PTT is seen.
    Lupus Anticoagulant
  33. What are the clinical complications of inhibitor formation?
    • Short half-life
    • No hemostatic ability
  34. What disease causes an activaiton of the hemostatic system, and leads to consumption of coagulation factors and platelets. This is a thrombotic mechanism, but causes bleeding.
    This disease is a thrombo-hemorrhagic complication secondary to an underlying disease process. It is manifested with uncontrolled and excessive thrombin generation and usually fibrinolytic activity.
    Disseminated Intravascular Coagulation (DIC)
  35. Venous thrombosis is _________ based, while arterial thrombosis is __________-derived.
    • Coagulation
    • Platelet
  36. What are the two types of systemic hypercoagulability?
    • DIC
    • Primary Fibrinolysis
  37. What are the confirmatory tests for DIC?
    • FSP -- fibrin split products
    • D-dimer
  38. What is the main treatment for DIC?
    Remove the underlying cause: DIC usually resolves itself after primary cause is removed
  39. Coagulation and fibrinolytic factors are produced by the fetal liver at ____ weeks gestation.
    10
  40. What do endothelial cells produce?
    • Factor VIII
    • vWF
    • tPA
    • PAI-1
  41. Platelets are produced at ____ weeks gestation
    11
  42. Endothelial cell function appears as soon as what are formed?
    Blood Vessels
  43. 25% of what factor is normal for newborns?
    FIX
  44. The fetus and newborn have an increased risk for what, due to the fact that Proteins C & S and factors V & VIII are not in balance (the hemostatic system in not in balance)?
    Thrombosis
  45. What disease is caused by a lack of vitamin K to produce normal levels of coagulation factors? This can cause major bleeds including intracranial bleeding. It can be seen in sick pre-term infants and with use of antibiotics in newborns. Why is this rarely seen in the US, and what is the treatment?
    • Hemorrhagic Disease of the Newborn
    • Because almost all newborns in the US receive oral Vitamin K
    • Treatment is Vitamin K supplementation
  46. How does the body compensate for the bleeding potential in pregnancy?
    • Major Changes in Coagulation System
    • Minor Changes in Platelet Function
    • Majro Changes in Fibrinolytic System
  47. There is a 6-fold increase for the risk of developing what during pregnancy?
    Thrombosis
  48. There is a high correlation between Cancer and what?
    Venous Thrombosis (VTE)
  49. Cancer patients have a 3-fold increased risk of developing a recurrent _______ as compared to a non-cancer population.
    VTE

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