W1-15 Bleeding disorders
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Questions and Answers

Which of the following is NOT a feature of Heparin-Induced Thrombocytopenia (HIT)?

  • Thrombosis in large arteries and veins
  • Platelet activation and aggregation
  • Mild thrombocytopenia (correct)
  • Formation of HMWH+PF4+antibody complexes
  • In clotting factor abnormalities, what is a common feature of Hemophilia A and Hemophilia B?

  • Increased protein C and S levels
  • Normal prothrombin time (PT)
  • Normal plasma level of the affected factor
  • Normal partial thromboplastin time (PTT) (correct)
  • What is the characteristic laboratory finding in Vitamin K deficiency?

  • Decreased factor VII level (correct)
  • Normal bleeding time (BT)
  • Increased platelet count (PC)
  • Decreased prothrombin time (PT)
  • Which factor deficiency is commonly seen in Vitamin K deficiency?

    <p>Factor II</p> Signup and view all the answers

    What is the main physiological role of vitamin K in coagulation?

    <p>Activating protein C and S</p> Signup and view all the answers

    What type of genetic disorder is von Willebrand disease?

    <p>Autosomal dominant</p> Signup and view all the answers

    Which condition starts with excessive coagulation in microvasculature and finishes with consumption of platelets and clotting factors followed by bleeding?

    <p>Disseminated Intravascular Coagulation (DIC)</p> Signup and view all the answers

    Which factor is deficient in Hemophilia A?

    <p>Factor VIII</p> Signup and view all the answers

    What is a characteristic clinical feature of Clotting Factors Abnormalities?

    <p><strong>Low</strong> PT and/or <strong>high</strong> PTT depending on the cascade involved</p> Signup and view all the answers

    What is the primary difference in laboratory evaluations between von Willebrand disease and disseminated intravascular coagulation (DIC)?

    <p>Reduced ADAMTS13 activity in von Willebrand disease, increased ADAMTS13 activity in DIC</p> Signup and view all the answers

    Which clinical manifestation is commonly shared between thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS)?

    <p>Renal manifestations such as oliguria and proteinuria</p> Signup and view all the answers

    What is a distinguishing morphologic change seen in microangiopathic hemolytic anemia (MAHA) associated with TTP and HUS?

    <p>Presence of schistocytes</p> Signup and view all the answers

    What is the key laboratory finding that helps differentiate disseminated intravascular coagulation (DIC) from thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS)?

    <p>Prolonged PT (prothrombin time) in DIC</p> Signup and view all the answers

    Which clotting factor abnormality is characterized by a deficiency of von Willebrand factor and factor VIII?

    <p>Hemophilia A</p> Signup and view all the answers

    What is the primary pathogenesis of Hemolytic Uremic Syndrome (HUS)?

    <p>Thrombotic microangiopathy</p> Signup and view all the answers

    Which condition is associated with qualitative platelet abnormalities and presents with a deficiency of glycoprotein IIb/IIIa or alpha granules?

    <p>Glanzmann thrombasthenia</p> Signup and view all the answers

    What is the classic triad of symptoms in a patient with Disseminated Intravascular Coagulation (DIC)?

    <p>Purpura, petechiae, ecchymoses</p> Signup and view all the answers

    Which condition is characterized by a deficiency of ADAMTS13 leading to the accumulation of unusually large von Willebrand factor multimers?

    <p>Thrombotic Thrombocytopenic Purpura (TTP)</p> Signup and view all the answers

    What is the primary etiology of Hemophilia B?

    <p>Deficiency of factor IX</p> Signup and view all the answers

    Which vascular wall abnormality is associated with small vessel vasculitis and presents with palpable purpura, arthralgia, and abdominal pain?

    <p>Henoch-Schönlein purpura</p> Signup and view all the answers

    Which condition is characterized by a deficiency of clotting factors due to vitamin K malabsorption or malnutrition?

    <p><strong>Vitamin K deficiency</strong></p> Signup and view all the answers

    What are the characteristic features of hemorrhagic diathesis associated with Scurvy?

    <p><strong>Gingival bleeding</strong>, <strong>perifollicular hemorrhages</strong>, <strong>ecchymoses</strong></p> Signup and view all the answers

    Hereditary Hemorrhagic Telangiectasia (Weber-Osler-Rendu syndrome) primarily affects which structures in the body?

    <p><strong>Blood vessels</strong></p> Signup and view all the answers

    What is the primary etiology of von Willebrand disease?

    <p>Mutated vWF gene</p> Signup and view all the answers

    Which of the following is a characteristic laboratory finding in von Willebrand disease?

    <p>Abnormal ristocetin test</p> Signup and view all the answers

    Which process leads to hemorrhagic diathesis and massive bleeding in disseminated intravascular coagulation (DIC)?

    <p>Widespread coagulation in microcirculation</p> Signup and view all the answers

    Which laboratory test is typically abnormal in disseminated intravascular coagulation (DIC)?

    <p>Elevated D-dimers</p> Signup and view all the answers

    What is a common leading manifestation of DIC?

    <p>Petechiae and purpura</p> Signup and view all the answers

    Which organ is mostly affected by DIC-related thrombosis?

    <p>Kidneys</p> Signup and view all the answers

    What is the primary morphologic change seen in DIC?

    <p>Fibrin microthrombi</p> Signup and view all the answers

    What type of anemia is commonly associated with DIC?

    <p>Microangiopathic hemolytic anemia</p> Signup and view all the answers

    Which of the following is an important feature of von Willebrand disease?

    <p>Inadequate stabilization by defective vWF</p> Signup and view all the answers

    What is the primary clinical manifestation of von Willebrand disease?

    <p>Bleeding from mucosal membranes and IV sites</p> Signup and view all the answers

    Which factor is deficient in von Willebrand disease?

    <p>Factor VIII</p> Signup and view all the answers

    In what setting does disseminated intravascular coagulation (DIC) commonly occur as a complication?

    <p>Obstetric pathologic conditions</p> Signup and view all the answers

    Which of the following quantitative platelet abnormalities is characterized by a self-limited condition and supportive treatment is beneficial?

    <p>Hemolytic-Uremic Syndrome (HUS)</p> Signup and view all the answers

    Which quantitative platelet abnormality is associated with a pentad of clinical manifestations including fever, hemorrhagic diathesis, MAHA, acute kidney injury, and CNS abnormalities?

    <p>TTP</p> Signup and view all the answers

    Which condition is characterized by schistocytes in the peripheral blood smear and thrombi in the hilar glomerular vessels?

    <p>TTP</p> Signup and view all the answers

    Which quantitative platelet abnormality involves the production of anti-platelet antibodies against GPIIb/IIIa and/or GPIb-IX?

    <p>ITP</p> Signup and view all the answers

    Which condition is more common in children and is characterized by E.coli (O157:H7) infection as an etiology?

    <p>HUS</p> Signup and view all the answers

    Which quantitative platelet abnormality involves the release of Shiga-like toxin leading to endothelial damage and formation of platelet microthrombi?

    <p>HUS</p> Signup and view all the answers

    Which condition has a prognosis that requires immediate intervention as it is life-threatening?

    <p>TTP</p> Signup and view all the answers

    Which quantitative platelet abnormality has laboratory evaluations showing decreased platelet count, increased bleeding time, normal prothrombin time, and normal partial thromboplastin time?

    <p>TTP</p> Signup and view all the answers

    Which condition involves clearance of immune complexes by splenic macrophages leading to thrombocytopenia?

    <p>ITP</p> Signup and view all the answers

    Which quantitative platelet abnormality may require splenectomy in refractory cases?

    <p>ITP</p> Signup and view all the answers

    Which condition presents with manifestations such as petechiae, purpura, ecchymoses, and intracranial hemorrhages in severe cases?

    <p>ITP</p> Signup and view all the answers

    Which condition has bone marrow evaluations showing an increased number of megakaryocytes and their immaturity as well as abnormally large platelets in the peripheral blood smear?

    <p>ITP</p> Signup and view all the answers

    What is the major manifestation of hemorrhagic diathesis?

    <p>Petechiae</p> Signup and view all the answers

    What is the classification of bleeding disorders due to vessel wall abnormalities?

    <p>Infections, small vessel vasculitides, collagen defects, and hereditary hemorrhagic teleangiectasia</p> Signup and view all the answers

    What is the laboratory finding in quantitative platelet disorders?

    <p>Decreased platelet count (PC)</p> Signup and view all the answers

    Which disorder is characterized by the production of antibodies against vWF-cleaving protease?

    <p>Thrombotic thrombocytopenic purpura (TTP)</p> Signup and view all the answers

    What is the major clinical feature of thrombotic thrombocytopenic purpura (TTP)?

    <p>Spontaneous platelet activation and aggregation</p> Signup and view all the answers

    Which condition is associated with microangiopathic hemolytic anemia (MAHA)?

    <p>Hemolytic uremic syndrome (HUS)</p> Signup and view all the answers

    What is the characteristic laboratory finding in qualitative platelet disorders?

    <p>Normal PT and PTT</p> Signup and view all the answers

    What is the classification of bleeding disorders due to clotting factors abnormalities?

    <p>Primary plug formation and secondary plug formation</p> Signup and view all the answers

    (TTP) Thrombotic thrombocytopenic purpura develops as an abnormal immune response common in which demographic?

    <p>common, affects adult women</p> Signup and view all the answers

    What is the primary pathogenic process involved in the development of TTP?

    <p>Formation of antibodies against ADAMTS13</p> Signup and view all the answers

    Study Notes

    • Hemorrhagic diathesis is characterized by regular and multiple bleeding, with major manifestations including mucosal bleeding, skin bleeding, and intraarticular and intracranial bleeding.
    • Hemorrhagic diathesis is associated with various vascular wall abnormalities, including meningococcemia, bacterial endocarditis, microscopic polyangiitis, Henoch-Schönlein purpura, scurvy, Ehlers-Danlos syndrome, hereditary hemorrhagic teleangiectasia (Weber-Osler-Rendu syndrome), vascular amyloidosis, and infections.
    • Hemorrhagic diathesis presents clinically with petechiae, purpura, ecchymoses, and hemarthroses, and laboratory tests show normal platelet count, bleeding time, prothrombin time, and partial thromboplastin time.
    • Bleeding disorders can be classified into those due to vessel wall abnormalities, platelet abnormalities, and clotting factor abnormalities, as well as mixed disorders.
    • Vascular wall abnormalities leading to hemorrhagic diathesis include infections, small vessel vasculitides, collagen defects, and hereditary hemorrhagic teleangiectasia.
    • Quantitative platelet abnormalities include thrombocytopenias with microangiopathic hemolytic anemia (MAHA), thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), immune thrombocytopenia purpura (ITP), drug-induced thrombocytopenia, heparin-induced thrombocytopenia, and HIV-associated thrombocytopenia.
    • Qualitative platelet abnormalities include Bernard-Soulier syndrome and Glanzmann thrombasthenia.
    • Thrombotic thrombocytopenic purpura (TTP) is a quantitative platelet disorder characterized by antibodies against vWF-cleaving protease (ADAMTS13), inability to cleave active vWF multimers into inactive vWF monomers, accumulation of (active) vWF multimers in the blood, spontaneous platelet activation and aggregation, formation of platelet microthrombi, consumption thrombocytopenia, hemorrhagic diathesis, microangiopathic hemolytic anemia, fever, acute kidney injury, and CNS abnormalities.
    • Hemolytic uremic syndrome (HUS) is a quantitative platelet disorder caused by E.coli (O157:H7) infection, endothelial damage, formation of platelet microthrombi, consumption thrombocytopenia, hemorrhagic diathesis, and microangiopathic hemolytic anemia.
    • Platelet disorders can be further classified into clinical and laboratory manifestations, including clinical features of hemorrhagic diathesis and laboratory findings, such as thrombocytopenia, bleeding time, prothrombin time, and partial thromboplastin time.
    • Thrombotic thrombocytopenic purpura (TTP) is characterized by decreased platelet count, prolonged bleeding time, normal prothrombin time and partial thromboplastin time, and microangiopathic hemolytic anemia, while hemolytic uremic syndrome (HUS) shows normal platelet count, prolonged bleeding time, and microangiopathic hemolytic anemia.
    • Thrombotic thrombocytopenic purpura (TTP) requires immediate intervention as it is a life-threatening condition, while hemolytic uremic syndrome (HUS) has a better prognosis with supportive care and adequate hydration.

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    Description

    Test your knowledge on the etiology, pathogenesis, morphologic changes, clinical manifestations, laboratory evaluations, and prognosis of quantitative platelet abnormalities, specifically focusing on ITP and an increased number of megakaryocytes in bone marrow.

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