99 Questions
Which of the following is the characteristic pathologic feature of Thrombotic Thrombocytopenic Purpura (TTP)?
Hyaline (platelet rich) microthrombi
Which laboratory finding is common in both TTP and Hemolytic Uremic Syndrome (HUS)?
Elevated bleeding time
Which of the following is the primary treatment for Thrombotic Thrombocytopenic Purpura (TTP)?
Plasma exchange
Which of the following is the most common age group affected by Thrombotic Thrombocytopenic Purpura (TTP)?
3rd-4th decade
Which of the following is the characteristic pathologic feature of Hemolytic Uremic Syndrome (HUS)?
Platelet microaggregates (hyaline microthrombi) in the glomerular capillaries
Which of the following is the most common causative agent in Hemolytic Uremic Syndrome (HUS)?
Escherichia coli O157:H7
Which of the following is the typical clinical presentation of Hemolytic Uremic Syndrome (HUS)?
Thrombocytopenia, microangiopathic hemolytic anemia, and acute renal failure
Which of the following is the most appropriate treatment for Hemolytic Uremic Syndrome (HUS)?
Conservative management (dialysis, antihypertensive, etc.)
Which of the following is a common laboratory finding in both Thrombotic Thrombocytopenic Purpura (TTP) and Hemolytic Uremic Syndrome (HUS)?
Microangiopathic hemolytic anemia
Which of the following is the key difference between Thrombotic Thrombocytopenic Purpura (TTP) and Hemolytic Uremic Syndrome (HUS)?
The age group affected
What is the most common bleeding site in patients with hemophilia A?
Joints
Which of the following is a characteristic feature of hemophilia A?
Bleeding episodes are mild, moderate or severe depending on the factor VIII level
What is the role of von Willebrand factor in hemophilia A?
Von Willebrand factor circulates bound to factor VIII and stabilizes it
What is the typical laboratory finding in hemophilia A?
Prolonged activated partial thromboplastin time (aPTT)
What is the most appropriate treatment for hemophilia A?
Replacement therapy with factor VIII concentrate or recombinant factor VIII
Which of the following is a characteristic of the inheritance pattern of hemophilia A?
It is inherited as an X-linked recessive trait
What is the primary function of the Partial Thromboplastin Time (PTT) test?
Assesses intrinsic clotting pathway activity
Which clotting factors affect both Partial Thromboplastin Time (PTT) and Prothrombin Time (PT)?
Prothrombin (II) and Fibrinogen (I)
Which symptom is NOT commonly associated with patients having problems in primary hemostasis?
Hepatomegaly
What clinical presentation is typical for patients with primary hemostasis issues?
Intracranial bleeding with severe thrombocytopenia
Which laboratory test measures the final step in the clotting cascade?
Thrombin time (TT)
Which of the following is a feature of primary hemostasis problems?
Epistaxis and GI bleeding
What distinguishes von Willebrand disease (vWD) from classic Hemophilia A?
Response to Desmopressin (DDAVP)
Which type of von Willebrand disease (vWD) is the most common?
Type 1
What is a characteristic laboratory finding in Bernard Soulier syndrome?
Normal platelet aggregation in response to vWF
How do individuals with von Willebrand disease (vWD) typically respond to Desmopressin (DDAVP)?
Increased vWF and factor VIII levels
What distinguishes the bleeding manifestations between classic Hemophilia A and von Willebrand disease?
Type of hemorrhage
What distinguishes Bernard Soulier syndrome from other platelet disorders?
Platelet aggregation in response to vWF
What is the primary defect in Glanzmann's thrombasthenia?
Genetic GIIB/IIIA deficiency leading to impaired platelet aggregation
What is the most common inherited cause of hypercoagulability?
Factor V Leiden mutation
At what platelet count level can fatal CNS or GI hemorrhage occur?
Around 5x10^3/µl
What is the characteristic of thrombocytopenia?
Prolonged bleeding time with normal PT and PTT
What is the typical clinical presentation of acute idiopathic (childhood) thrombocytopenic purpura (ITP)?
Develops acutely within 1-2 weeks with a short duration
At what platelet count level may bleeding occur, depending on the platelets' functional status?
Between 20-60x10^3/µl
Which of the following is the most common cause of chronic Immune Thrombocytopenic Purpura (ITP) in adults?
Idiopathic (unknown cause)
What is the characteristic finding on peripheral blood smear in Immune Thrombocytopenic Purpura (ITP)?
Large platelets
Which of the following is the most common initial treatment for severe cases of Immune Thrombocytopenic Purpura (ITP)?
High-dose intravenous immunoglobulins
What is the underlying mechanism in Immune Thrombocytopenic Purpura (ITP)?
Formation of anti-platelet antibodies
Which of the following is the characteristic bone marrow finding in Immune Thrombocytopenic Purpura (ITP)?
Increased megakaryocyte numbers
What is the underlying pathology in microangiopathic thrombocytopenia?
Formation of platelet microthrombi in small blood vessels
What is the most common bleeding site in patients with hemophilia A?
Muscles and joints
What is the primary defect in Glanzmann's thrombasthenia?
Deficiency in platelet glycoprotein IIb/IIIa complex
What is the most appropriate treatment for hemophilia A?
Factor VIII concentrate or recombinant VIII replacement therapy
Which type of von Willebrand disease (vWD) is the most common?
Type 1
What is the role of von Willebrand factor in hemophilia A?
It stabilizes and protects factor VIII from degradation
What is the typical laboratory finding in hemophilia A?
Prolonged activated partial thromboplastin time (aPTT)
What is the most common type of von Willebrand disease (vWD)?
Type 1
Which of the following is NOT a characteristic of von Willebrand disease (vWD)?
Hemarthroses and deep muscle hemorrhage
How do individuals with von Willebrand disease (vWD) typically respond to Desmopressin (DDAVP) treatment?
DDAVP temporarily increases vWF and factor VIII levels 2-3 times
What is the characteristic laboratory finding in Bernard Soulier syndrome?
All of the above
Which of the following is a distinguishing feature between classic Hemophilia A and von Willebrand disease (vWD)?
All of the above
Which type of von Willebrand disease (vWD) has the most severe bleeding?
Type 3
What is the purpose of the bleeding time test?
To assess platelet function by timing how long it takes for bleeding to stop
Which of the following is a common symptom of patients with primary hemostasis problems?
Hematuria (blood in urine)
What is measured by the Prothrombin Time (PT) and International Normalized Ratio (INR) tests?
Activity of the extrinsic clotting pathway
Which of the following is a common skin manifestation in patients with primary hemostasis problems?
Ecchymoses (larger than 1cm bleeding spots)
Which of the following clotting factors affect both the Partial Thromboplastin Time (PTT) and Prothrombin Time (PT)?
Factor X, Factor V, Prothrombin (Factor II), and Fibrinogen (Factor I)
Which laboratory test measures the final step in the clotting cascade, where fibrinogen is converted to fibrin?
Thrombin Time (TT)
What is the most common cause of chronic Immune Thrombocytopenic Purpura (ITP) in adults?
Idiopathic or secondary to conditions like SLE or HIV
What is the characteristic finding on peripheral blood smear in Immune Thrombocytopenic Purpura (ITP)?
Large platelets
Which of the following is the most common initial treatment for severe cases of Immune Thrombocytopenic Purpura (ITP)?
High-dose intravenous immunoglobulins
What is the underlying mechanism in Immune Thrombocytopenic Purpura (ITP)?
Formation of anti-platelet antibodies, primarily IgG against GPIIb/IIIa
Which of the following is the characteristic bone marrow finding in Immune Thrombocytopenic Purpura (ITP)?
Increased megakaryocytes
What is the underlying pathology in microangiopathic thrombocytopenia?
Platelet microthrombi formation in small blood vessels
What is a key difference between Hemolytic Uremic Syndrome (HUS) and Thrombotic Thrombocytopenic Purpura (TTP)?
TTP commonly presents with neurological symptoms, while HUS is limited to kidney involvement.
What distinguishes Disseminated Intravascular Coagulation (DIC) from Hemolytic Uremic Syndrome (HUS)?
HUS is a primary disease, whereas DIC is a complication of conditions with endothelial cell damage.
What is a typical laboratory finding in patients with Disseminated Intravascular Coagulation (DIC)?
Elevated D-dimers and other fibrin degradation products
Which of the following statements regarding Hemolytic Uremic Syndrome (HUS) is accurate?
HUS can follow viral or bacterial infections and predominantly affects children.
What distinguishes Disseminated Intravascular Coagulation (DIC) from Thrombotic Thrombocytopenic Purpura (TTP)?
DIC leads to thrombo-hemorrhagic disorder, while TTP does not involve bleeding.
Which laboratory finding is characteristic of patients with Disseminated Intravascular Coagulation (DIC)?
Low levels of clotting factors
Which coagulation factor is deficient in individuals with hemophilia B?
Factor IX
What is the most common inheritance pattern of von Willebrand disease (vWD)?
Autosomal dominant
What is the role of von Willebrand factor (vWF) in the coagulation process?
It stabilizes factor VIII
Which laboratory test is used to diagnose von Willebrand disease (vWD)?
Ristocetin-induced platelet aggregation (RIPA)
What is the most common initial treatment for severe cases of hemophilia B?
Recombinant factor IX concentrates
Which of the following is a characteristic of the inheritance pattern of hemophilia B?
X-linked recessive
What is the primary role of factor VIII in the coagulation cascade?
Activates factor X, which then activates prothrombin
Which of the following is a characteristic clinical manifestation of hemophilia A?
Hemarthrosis and muscle hematomas
What is the underlying genetic defect in hemophilia A?
X-linked recessive mutation in the factor VIII gene
Which laboratory test is typically prolonged in patients with hemophilia A?
Partial thromboplastin time (PTT)
What is the primary treatment for hemophilia A?
Factor VIII replacement therapy
Which of the following statements is true regarding the inheritance pattern of hemophilia A?
It is an X-linked recessive disorder that almost exclusively affects males
What is the underlying mechanism in Immune Thrombocytopenic Purpura (ITP)?
Formation of anti-platelet antibodies, IgG against platelet glycoprotein IIb/IIIa
What is the underlying pathology in microangiopathic thrombocytopenia?
Formation of platelet microthrombi in small blood vessels that mechanically damage red blood cells
Which of the following is the most common initial treatment for severe cases of Immune Thrombocytopenic Purpura (ITP)?
Corticosteroids
What is the characteristic laboratory finding on peripheral blood smear in Immune Thrombocytopenic Purpura (ITP)?
Increased numbers of large platelets
What is the underlying cause of chronic Immune Thrombocytopenic Purpura (ITP) in adults?
Autoimmune disorders like systemic lupus erythematosus (SLE)
What is the characteristic bone marrow finding in Immune Thrombocytopenic Purpura (ITP)?
Increased number of megakaryocytes
What is the most common causative agent in Hemolytic Uremic Syndrome (HUS)?
Toxin-induced endothelial damage
Which laboratory test measures the final step in the clotting cascade, where fibrinogen is converted to fibrin?
Prothrombin Time (PT)
What distinguishes Hemolytic Uremic Syndrome (HUS) from Thrombotic Thrombocytopenic Purpura (TTP)?
E.coli O157:H7 as a causative agent
What distinguishes the bleeding manifestations between Thrombotic Thrombocytopenic Purpura (TTP) and Hemolytic Uremic Syndrome (HUS)?
Platelet microaggregate formation
Which laboratory finding is common in both Hemolytic Uremic Syndrome (HUS) and Thrombotic Thrombocytopenic Purpura (TTP)?
Normal Prothrombin Time (PT)
Which of the following is a key distinguishing feature between von Willebrand disease (vWD) and classic hemophilia A?
All of the above.
Which of the following statements about von Willebrand disease (vWD) types is correct?
Type 3 vWD is the most severe form and patients do not respond to desmopressin (DDAVP) treatment.
Which of the following statements regarding the treatment of von Willebrand disease (vWD) is correct?
Desmopressin (DDAVP) can be used to temporarily increase vWF and factor VIII levels 2-3 times in patients with Type 1 and Type 2 vWD.
Which of the following laboratory findings is characteristic of Bernard-Soulier syndrome?
All of the above
Which of the following statements regarding the pathophysiology of Immune Thrombocytopenic Purpura (ITP) is correct?
In ITP, autoantibodies bind to and destroy platelets, resulting in thrombocytopenia.
Which of the following statements regarding the laboratory findings in Disseminated Intravascular Coagulation (DIC) is correct?
Patients with DIC typically exhibit thrombocytopenia and the presence of schistocytes on peripheral blood smear.
This quiz covers the initial laboratory tests for bleeding abnormalities including platelet count, bleeding time, partial thromboplastin time (PTT), and prothrombin time (PT)/INR. Test your knowledge on how these tests are used to assess coagulation disorders.
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