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Questions and Answers
Which of the following is NOT a feature of Heparin-Induced Thrombocytopenia (HIT)?
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?
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?
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?
Which factor deficiency is commonly seen in Vitamin K deficiency?
What is the main physiological role of vitamin K in coagulation?
What is the main physiological role of vitamin K in coagulation?
What type of genetic disorder is von Willebrand disease?
What type of genetic disorder is von Willebrand disease?
Which condition starts with excessive coagulation in microvasculature and finishes with consumption of platelets and clotting factors followed by bleeding?
Which condition starts with excessive coagulation in microvasculature and finishes with consumption of platelets and clotting factors followed by bleeding?
Which factor is deficient in Hemophilia A?
Which factor is deficient in Hemophilia A?
What is a characteristic clinical feature of Clotting Factors Abnormalities?
What is a characteristic clinical feature of Clotting Factors Abnormalities?
What is the primary difference in laboratory evaluations between von Willebrand disease and disseminated intravascular coagulation (DIC)?
What is the primary difference in laboratory evaluations between von Willebrand disease and disseminated intravascular coagulation (DIC)?
Which clinical manifestation is commonly shared between thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS)?
Which clinical manifestation is commonly shared between thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS)?
What is a distinguishing morphologic change seen in microangiopathic hemolytic anemia (MAHA) associated with TTP and HUS?
What is a distinguishing morphologic change seen in microangiopathic hemolytic anemia (MAHA) associated with TTP and HUS?
What is the key laboratory finding that helps differentiate disseminated intravascular coagulation (DIC) from thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS)?
What is the key laboratory finding that helps differentiate disseminated intravascular coagulation (DIC) from thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS)?
Which clotting factor abnormality is characterized by a deficiency of von Willebrand factor and factor VIII?
Which clotting factor abnormality is characterized by a deficiency of von Willebrand factor and factor VIII?
What is the primary pathogenesis of Hemolytic Uremic Syndrome (HUS)?
What is the primary pathogenesis of Hemolytic Uremic Syndrome (HUS)?
Which condition is associated with qualitative platelet abnormalities and presents with a deficiency of glycoprotein IIb/IIIa or alpha granules?
Which condition is associated with qualitative platelet abnormalities and presents with a deficiency of glycoprotein IIb/IIIa or alpha granules?
What is the classic triad of symptoms in a patient with Disseminated Intravascular Coagulation (DIC)?
What is the classic triad of symptoms in a patient with Disseminated Intravascular Coagulation (DIC)?
Which condition is characterized by a deficiency of ADAMTS13 leading to the accumulation of unusually large von Willebrand factor multimers?
Which condition is characterized by a deficiency of ADAMTS13 leading to the accumulation of unusually large von Willebrand factor multimers?
What is the primary etiology of Hemophilia B?
What is the primary etiology of Hemophilia B?
Which vascular wall abnormality is associated with small vessel vasculitis and presents with palpable purpura, arthralgia, and abdominal pain?
Which vascular wall abnormality is associated with small vessel vasculitis and presents with palpable purpura, arthralgia, and abdominal pain?
Which condition is characterized by a deficiency of clotting factors due to vitamin K malabsorption or malnutrition?
Which condition is characterized by a deficiency of clotting factors due to vitamin K malabsorption or malnutrition?
What are the characteristic features of hemorrhagic diathesis associated with Scurvy?
What are the characteristic features of hemorrhagic diathesis associated with Scurvy?
Hereditary Hemorrhagic Telangiectasia (Weber-Osler-Rendu syndrome) primarily affects which structures in the body?
Hereditary Hemorrhagic Telangiectasia (Weber-Osler-Rendu syndrome) primarily affects which structures in the body?
What is the primary etiology of von Willebrand disease?
What is the primary etiology of von Willebrand disease?
Which of the following is a characteristic laboratory finding in von Willebrand disease?
Which of the following is a characteristic laboratory finding in von Willebrand disease?
Which process leads to hemorrhagic diathesis and massive bleeding in disseminated intravascular coagulation (DIC)?
Which process leads to hemorrhagic diathesis and massive bleeding in disseminated intravascular coagulation (DIC)?
Which laboratory test is typically abnormal in disseminated intravascular coagulation (DIC)?
Which laboratory test is typically abnormal in disseminated intravascular coagulation (DIC)?
What is a common leading manifestation of DIC?
What is a common leading manifestation of DIC?
Which organ is mostly affected by DIC-related thrombosis?
Which organ is mostly affected by DIC-related thrombosis?
What is the primary morphologic change seen in DIC?
What is the primary morphologic change seen in DIC?
What type of anemia is commonly associated with DIC?
What type of anemia is commonly associated with DIC?
Which of the following is an important feature of von Willebrand disease?
Which of the following is an important feature of von Willebrand disease?
What is the primary clinical manifestation of von Willebrand disease?
What is the primary clinical manifestation of von Willebrand disease?
Which factor is deficient in von Willebrand disease?
Which factor is deficient in von Willebrand disease?
In what setting does disseminated intravascular coagulation (DIC) commonly occur as a complication?
In what setting does disseminated intravascular coagulation (DIC) commonly occur as a complication?
Which of the following quantitative platelet abnormalities is characterized by a self-limited condition and supportive treatment is beneficial?
Which of the following quantitative platelet abnormalities is characterized by a self-limited condition and supportive treatment is beneficial?
Which quantitative platelet abnormality is associated with a pentad of clinical manifestations including fever, hemorrhagic diathesis, MAHA, acute kidney injury, and CNS abnormalities?
Which quantitative platelet abnormality is associated with a pentad of clinical manifestations including fever, hemorrhagic diathesis, MAHA, acute kidney injury, and CNS abnormalities?
Which condition is characterized by schistocytes in the peripheral blood smear and thrombi in the hilar glomerular vessels?
Which condition is characterized by schistocytes in the peripheral blood smear and thrombi in the hilar glomerular vessels?
Which quantitative platelet abnormality involves the production of anti-platelet antibodies against GPIIb/IIIa and/or GPIb-IX?
Which quantitative platelet abnormality involves the production of anti-platelet antibodies against GPIIb/IIIa and/or GPIb-IX?
Which condition is more common in children and is characterized by E.coli (O157:H7) infection as an etiology?
Which condition is more common in children and is characterized by E.coli (O157:H7) infection as an etiology?
Which quantitative platelet abnormality involves the release of Shiga-like toxin leading to endothelial damage and formation of platelet microthrombi?
Which quantitative platelet abnormality involves the release of Shiga-like toxin leading to endothelial damage and formation of platelet microthrombi?
Which condition has a prognosis that requires immediate intervention as it is life-threatening?
Which condition has a prognosis that requires immediate intervention as it is life-threatening?
Which quantitative platelet abnormality has laboratory evaluations showing decreased platelet count, increased bleeding time, normal prothrombin time, and normal partial thromboplastin time?
Which quantitative platelet abnormality has laboratory evaluations showing decreased platelet count, increased bleeding time, normal prothrombin time, and normal partial thromboplastin time?
Which condition involves clearance of immune complexes by splenic macrophages leading to thrombocytopenia?
Which condition involves clearance of immune complexes by splenic macrophages leading to thrombocytopenia?
Which quantitative platelet abnormality may require splenectomy in refractory cases?
Which quantitative platelet abnormality may require splenectomy in refractory cases?
Which condition presents with manifestations such as petechiae, purpura, ecchymoses, and intracranial hemorrhages in severe cases?
Which condition presents with manifestations such as petechiae, purpura, ecchymoses, and intracranial hemorrhages in severe cases?
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?
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?
What is the major manifestation of hemorrhagic diathesis?
What is the major manifestation of hemorrhagic diathesis?
What is the classification of bleeding disorders due to vessel wall abnormalities?
What is the classification of bleeding disorders due to vessel wall abnormalities?
What is the laboratory finding in quantitative platelet disorders?
What is the laboratory finding in quantitative platelet disorders?
Which disorder is characterized by the production of antibodies against vWF-cleaving protease?
Which disorder is characterized by the production of antibodies against vWF-cleaving protease?
What is the major clinical feature of thrombotic thrombocytopenic purpura (TTP)?
What is the major clinical feature of thrombotic thrombocytopenic purpura (TTP)?
Which condition is associated with microangiopathic hemolytic anemia (MAHA)?
Which condition is associated with microangiopathic hemolytic anemia (MAHA)?
What is the characteristic laboratory finding in qualitative platelet disorders?
What is the characteristic laboratory finding in qualitative platelet disorders?
What is the classification of bleeding disorders due to clotting factors abnormalities?
What is the classification of bleeding disorders due to clotting factors abnormalities?
(TTP) Thrombotic thrombocytopenic purpura develops as an abnormal immune response common in which demographic?
(TTP) Thrombotic thrombocytopenic purpura develops as an abnormal immune response common in which demographic?
What is the primary pathogenic process involved in the development of TTP?
What is the primary pathogenic process involved in the development of TTP?
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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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.