Questions and Answers
What is the expected clotting time for healthy plasma when a diluted thrombin solution is added?
Which condition is NOT typically classified under bleeding disorders?
Which factor is deficient in Hemophilia B?
What factor characterizes Hemophilia A?
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Which of the following is a hereditary bleeding disorder?
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Which of the following can lead to prolonged clotting time when plasma fibrinogen is low?
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What is the mode of inheritance for Hemophilia A?
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Which of the following is NOT a coagulopathy?
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What is a common cause of Thrombocytopenia related to increased platelet destruction?
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Which condition is characterized by a deficiency or malfunction of the vWBF/GPIb complex?
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What factor's deficiency is associated with thrombohemorrhagic events due to a throwing mutation?
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What common symptom might indicate Idiopathic Thrombocytopenic Purpura (ITP) in an adult?
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What is the peak age of incidence for Idiopathic Thrombocytopenic Purpura (ITP) in adults?
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Which medication is a reversal agent specific for factor Xa inhibitor anticoagulants?
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What is the expected laboratory finding in a patient with ITP, barring any secondary effects from bleeding?
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Which of the following conditions leads to qualitative platelet disorders?
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What role does vWF play in primary hemostasis?
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Which of the following statements about factor VIII and von Willebrand factor (vWF) is correct?
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What is the characteristic lab finding in von Willebrand disease (vWD)?
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Which treatment option is ineffective for Type 3 von Willebrand disease?
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Which bleeding disorder is characterized by spontaneous bleeding from telangiectasias and is hereditary?
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What is a common presentation of acquired purpura simplex?
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In the context of vWD, what does the treatment with antifibrinolytics aim to do?
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Which factor is primarily protected by vWF to prevent inactivation or degradation?
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Study Notes
Clotting Time in Anticoagulated Plasma
- Prolonged clotting time occurs with low fibrinogen levels or high fibrinolytic split products.
- Normal clotting time range: 12–14 seconds.
Bleeding Disorders
- Classification: Primary, secondary, or both.
- Von Willebrand Disease affects both primary and secondary hemostasis.
Coagulopathies
- Types of coagulopathies:
- Idiopathic
- Autoimmune
- Infections
- Neoplasms
- Medications
- Hereditary disorders such as Hemophilia A, B, C, and Von Willebrand Disease.
Hemophilia
- Inherited bleeding disorder due to deficiency in coagulation factors.
- Hemophilia A: Deficiency of Factor VIII, X-linked.
- Hemophilia B: Deficiency of Factor IX (Christmas disease), X-linked.
- Hemophilia C: Deficiency of Factor XI, autosomal recessive.
- 1 in 5,000 to 1 in 10,000 individuals affected; severe disease in two-thirds of cases.
- Gender distribution skewed towards males, but spontaneous mutations may occur in females.
Senile Purpura
- Result from progressive loss of dermis and vascular wall due to aging.
- Commonly appears on the dorsal side of hands and wrists, often triggered by venipuncture.
Hypercoagulability and Other Coagulopathies
- Factor V Leiden mutation leads to thrombohemorrhagic events.
- Antiphospholipid syndrome (Pr.C and Pr.S deficiency) triggers DIC.
- Thrombotic Thrombocytopenic Purpura (TTP) associated with large vWBF multimers.
Platelet Disorders
- Platelet disorders can be quantitative (thrombocytopenia) or qualitative (thrombasthenia).
- Causes of reduced platelet production: aplastic anemia, radiation, leukemia, drugs, and infections.
- Increased destruction causes include:
- Idiopathic Thrombocytopenic Purpura (ITP)
- Viral infections like HIV or malaria
- Lymphoma and Drug-induced HIT.
Thrombasthenias
- Common causes include:
- Von Willebrand Disease: deficiency/malfunction of vWF/GPIb complex.
- Bernard-Soulier syndrome: GPIb deficiency.
- Glanzmann’s thrombasthenia: GPIIb/IIIa deficiency.
Idiopathic Thrombocytopenic Purpura (ITP)
- Predominantly affects females (5:1 ratio).
- Peak incidence: ages 20-40 years; acute pediatric cases peak at 2-4 years.
- Remission occurs within 2-4 weeks.
- Symptoms include epistaxis, petechiae, or purpura.
- Normal white cell count and hemoglobin unless due to secondary anemia.
- Platelet antibody tests are often unreliable.
ITP Treatment
- Treatment generally starts only if severe thrombocytopenia is present.
- Idarucizumab used for reversal of anticoagulants.
- Andexanet alpha reverses factor Xa inhibitors like Rivaroxaban and Apixaban.
Functions of Von Willebrand Factor (vWF)
- Primary Hemostasis:
- Mediates adhesion of platelets to injured sites.
- Promotes aggregation at sites of injury.
- Secondary Hemostasis:
- Stabilizes and increases the half-life of coagulation factor VIII.
Laboratory Findings
- Normal platelet count for all types except 2B.
- Normal Prothrombin Time (PT).
- Activated Partial Thromboplastin Time (aPTT) is normal to elevated depending on factor VIII levels.
Screening Tests for vWD
- Plasma vWF antigen and activity levels.
- Factor VIII activity levels.
Treatments for Von Willebrand Disease (vWD)
- Desmopressin (DDAVP) for Type 1 and II (not effective for Type 2B).
- Replacement therapy using vWF concentrates.
- Antifibrinolytics and topical thrombin strategies.
Other Notable Conditions
- Hereditary Hemorrhagic Telangiectasia (HHT): Autosomal dominant disorder causing spontaneous bleeding from dilated vessels.
- Ehlers-Danlos syndrome and osteogenesis imperfecta linked to collagen defects leading to vessel fragility.
- Marfan syndrome associated with elastin issues.
- Acquired disorders include idiopathic purpura and increased fragility of skin vessels, typically presenting as easy bruising.
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Description
This quiz focuses on bleeding disorders such as Von Willebrand disease and various coagulopathies. It explores the effects of a diluted thrombin solution and the implications of clotting time alterations in relation to plasma fibrinogen levels and fibrinolytic split products. Test your knowledge of the underlying mechanisms and classifications of these disorders.
