57 Questions
Which of the following is NOT a feature of Heparin-Induced Thrombocytopenia (HIT)?
Mild thrombocytopenia
In clotting factor abnormalities, what is a common feature of Hemophilia A and Hemophilia B?
Normal partial thromboplastin time (PTT)
What is the characteristic laboratory finding in Vitamin K deficiency?
Decreased factor VII level
Which factor deficiency is commonly seen in Vitamin K deficiency?
Factor II
What is the main physiological role of vitamin K in coagulation?
Activating protein C and S
What type of genetic disorder is von Willebrand disease?
Autosomal dominant
Which condition starts with excessive coagulation in microvasculature and finishes with consumption of platelets and clotting factors followed by bleeding?
Disseminated Intravascular Coagulation (DIC)
Which factor is deficient in Hemophilia A?
Factor VIII
What is a characteristic clinical feature of Clotting Factors Abnormalities?
Low PT and/or high PTT depending on the cascade involved
What is the primary difference in laboratory evaluations between von Willebrand disease and disseminated intravascular coagulation (DIC)?
Reduced ADAMTS13 activity in von Willebrand disease, increased ADAMTS13 activity in DIC
Which clinical manifestation is commonly shared between thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS)?
Renal manifestations such as oliguria and proteinuria
What is a distinguishing morphologic change seen in microangiopathic hemolytic anemia (MAHA) associated with TTP and HUS?
Presence of schistocytes
What is the key laboratory finding that helps differentiate disseminated intravascular coagulation (DIC) from thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS)?
Prolonged PT (prothrombin time) in DIC
Which clotting factor abnormality is characterized by a deficiency of von Willebrand factor and factor VIII?
Hemophilia A
What is the primary pathogenesis of Hemolytic Uremic Syndrome (HUS)?
Thrombotic microangiopathy
Which condition is associated with qualitative platelet abnormalities and presents with a deficiency of glycoprotein IIb/IIIa or alpha granules?
Glanzmann thrombasthenia
What is the classic triad of symptoms in a patient with Disseminated Intravascular Coagulation (DIC)?
Purpura, petechiae, ecchymoses
Which condition is characterized by a deficiency of ADAMTS13 leading to the accumulation of unusually large von Willebrand factor multimers?
Thrombotic Thrombocytopenic Purpura (TTP)
What is the primary etiology of Hemophilia B?
Deficiency of factor IX
Which vascular wall abnormality is associated with small vessel vasculitis and presents with palpable purpura, arthralgia, and abdominal pain?
Henoch-Schönlein purpura
Which condition is characterized by a deficiency of clotting factors due to vitamin K malabsorption or malnutrition?
Vitamin K deficiency
What are the characteristic features of hemorrhagic diathesis associated with Scurvy?
Gingival bleeding, perifollicular hemorrhages, ecchymoses
Hereditary Hemorrhagic Telangiectasia (Weber-Osler-Rendu syndrome) primarily affects which structures in the body?
Blood vessels
What is the primary etiology of von Willebrand disease?
Mutated vWF gene
Which of the following is a characteristic laboratory finding in von Willebrand disease?
Abnormal ristocetin test
Which process leads to hemorrhagic diathesis and massive bleeding in disseminated intravascular coagulation (DIC)?
Widespread coagulation in microcirculation
Which laboratory test is typically abnormal in disseminated intravascular coagulation (DIC)?
Elevated D-dimers
What is a common leading manifestation of DIC?
Petechiae and purpura
Which organ is mostly affected by DIC-related thrombosis?
Kidneys
What is the primary morphologic change seen in DIC?
Fibrin microthrombi
What type of anemia is commonly associated with DIC?
Microangiopathic hemolytic anemia
Which of the following is an important feature of von Willebrand disease?
Inadequate stabilization by defective vWF
What is the primary clinical manifestation of von Willebrand disease?
Bleeding from mucosal membranes and IV sites
Which factor is deficient in von Willebrand disease?
Factor VIII
In what setting does disseminated intravascular coagulation (DIC) commonly occur as a complication?
Obstetric pathologic conditions
Which of the following quantitative platelet abnormalities is characterized by a self-limited condition and supportive treatment is beneficial?
Hemolytic-Uremic Syndrome (HUS)
Which quantitative platelet abnormality is associated with a pentad of clinical manifestations including fever, hemorrhagic diathesis, MAHA, acute kidney injury, and CNS abnormalities?
TTP
Which condition is characterized by schistocytes in the peripheral blood smear and thrombi in the hilar glomerular vessels?
TTP
Which quantitative platelet abnormality involves the production of anti-platelet antibodies against GPIIb/IIIa and/or GPIb-IX?
ITP
Which condition is more common in children and is characterized by E.coli (O157:H7) infection as an etiology?
HUS
Which quantitative platelet abnormality involves the release of Shiga-like toxin leading to endothelial damage and formation of platelet microthrombi?
HUS
Which condition has a prognosis that requires immediate intervention as it is life-threatening?
TTP
Which quantitative platelet abnormality has laboratory evaluations showing decreased platelet count, increased bleeding time, normal prothrombin time, and normal partial thromboplastin time?
TTP
Which condition involves clearance of immune complexes by splenic macrophages leading to thrombocytopenia?
ITP
Which quantitative platelet abnormality may require splenectomy in refractory cases?
ITP
Which condition presents with manifestations such as petechiae, purpura, ecchymoses, and intracranial hemorrhages in severe cases?
ITP
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?
ITP
What is the major manifestation of hemorrhagic diathesis?
Petechiae
What is the classification of bleeding disorders due to vessel wall abnormalities?
Infections, small vessel vasculitides, collagen defects, and hereditary hemorrhagic teleangiectasia
What is the laboratory finding in quantitative platelet disorders?
Decreased platelet count (PC)
Which disorder is characterized by the production of antibodies against vWF-cleaving protease?
Thrombotic thrombocytopenic purpura (TTP)
What is the major clinical feature of thrombotic thrombocytopenic purpura (TTP)?
Spontaneous platelet activation and aggregation
Which condition is associated with microangiopathic hemolytic anemia (MAHA)?
Hemolytic uremic syndrome (HUS)
What is the characteristic laboratory finding in qualitative platelet disorders?
Normal PT and PTT
What is the classification of bleeding disorders due to clotting factors abnormalities?
Primary plug formation and secondary plug formation
(TTP) Thrombotic thrombocytopenic purpura develops as an abnormal immune response common in which demographic?
common, affects adult women
What is the primary pathogenic process involved in the development of TTP?
Formation of antibodies against ADAMTS13
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.
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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