Question 1

Criteria for diagnosing Glanzmann's thrombasthenia include:

Accepted Answer

A)  An autosomal recessive trait with clinical manifestations expressed in homozygotes only 
B)  Prolonged bleeding time 
C)  Normal platelet count 
D)  Absence of platelet aggregation to ADP (thrombin, collagen, and epinephrine) 
E)  All of the above 
A)  An autosomal recessive trait with clinical manifestations expressed in homozygotes only 
B)  Prolonged bleeding time 
C)  Normal platelet count 
D)  Absence of platelet aggregation to ADP (thrombin, collagen, and epinephrine) 
E)  All of the above E

Question 2

Ineffective thrombopoiesis is defined as:

Accepted Answer

A)  The state in which the number of megakaryocytes in the bone marrow is normal to increased, but maturation and release is abnormal 
B)  The state in which the number of megakaryocytes in the bone marrow is decreased 
C)  The state in which the number of megakaryocytes in the bone marrow is normal to decreased, but maturation and release is normal 
D)  The state in which the number of thrombocytes in the bone marrow is normal to increased, but maturation and release is abnormal 
E)  None of the above 
A)  The state in which the number of megakaryocytes in the bone marrow is normal to increased, but maturation and release is abnormal 
B)  The state in which the number of megakaryocytes in the bone marrow is decreased 
C)  The state in which the number of megakaryocytes in the bone marrow is normal to decreased, but maturation and release is normal 
D)  The state in which the number of thrombocytes in the bone marrow is normal to increased, but maturation and release is abnormal 
E)  None of the above A

Question 3

Platelet transfusion administration for bleeding episodes in Glanzmann's thrombasthenia is associated with:

Accepted Answer

A)  Development of antibodies to GPIIb/IIIa 
B)  Development of paradoxical thrombocytopenia and worsening bleeding 
C)  Development of anti D antibodies 
D)  Arterial and venous thrombosis 
E)  All of the above 
A)  Development of antibodies to GPIIb/IIIa 
B)  Development of paradoxical thrombocytopenia and worsening bleeding 
C)  Development of anti D antibodies 
D)  Arterial and venous thrombosis 
E)  All of the above A

Question 4

All of the following are characteristic of hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome) except:

Accepted Answer

A)  Telangiectatic lesions occur on mucus membranes. 
B)  Iron deficiency anemia is usually present. 
C)  Abnormal platelet aggregation in response to thrombin is present. 
D)  Epistaxis is common. 
E)  An autosomal inheritance pattern is present. 
A)  Telangiectatic lesions occur on mucus membranes. 
B)  Iron deficiency anemia is usually present. 
C)  Abnormal platelet aggregation in response to thrombin is present. 
D)  Epistaxis is common. 
E)  An autosomal inheritance pattern is present.

Question 5

von Willebrand's disease (vWD) and Bernard-Soulier syndrome represent disorders of platelet __________.

Accepted Answer

A)  Aggregation 
B)  Adhesion 
C)  Secretion 
D)  Response 
E)  None of the above 
A)  Aggregation 
B)  Adhesion 
C)  Secretion 
D)  Response 
E)  None of the above

Question 6

The most important therapeutic intervention for suspected heparin-induced thrombocytopenia is:

Accepted Answer

A)  Warfarin 
B)  Lepthirudin (recombinant form of hirudin) 
C)  Dextran 
D)  Aspirin 
E)  Discontinuation of heparin 
A)  Warfarin 
B)  Lepthirudin (recombinant form of hirudin) 
C)  Dextran 
D)  Aspirin 
E)  Discontinuation of heparin

Question 7

__________ is a common clinical manifestation of the various paraproteinemias.

Accepted Answer

A)  Thromboembolism 
B)  Bleeding 
C)  Thyroid dysfunction 
D)  Disseminating intravascular coagulation (DI
C)  
E)  None of the above 
A)  Thromboembolism 
B)  Bleeding 
C)  Thyroid dysfunction 
D)  Disseminating intravascular coagulation (DI
C)  
E)  None of the above

Question 8

The malignant paraproteins result in:

Accepted Answer

A)  Coagulation abnormalities 
B)  Acquired circulating anticoagulants 
C)  Platelet dysfunction 
D)  All of the above 
E)  None of the above 
A)  Coagulation abnormalities 
B)  Acquired circulating anticoagulants 
C)  Platelet dysfunction 
D)  All of the above 
E)  None of the above

Question 9

Individuals with chronic liver disease are often associated with a significant hemorrhagic diathesis. Abnormal platelet function tests in these patients include all of the following except:

Accepted Answer

A)  Reduced platelet adhesion 
B)  Abnormal platelet aggregation with ristocetin 
C)  Abnormal PF3 availability 
D)  Abnormal platelet aggregation to thrombin 
E)  None of the above 
A)  Reduced platelet adhesion 
B)  Abnormal platelet aggregation with ristocetin 
C)  Abnormal PF3 availability 
D)  Abnormal platelet aggregation to thrombin 
E)  None of the above

Question 10

__________ is a rare autosomal recessive disorder of platelet function associated with an abnormality of membrane glycoproteins IIb and IIIa, causing decreased platelet aggregation.

Accepted Answer

A)  Bernard-Soulier syndrome 
B)  Glanzmann's thrombasthenia 
C)  von Willebrand's disease 
D)  Congenital afibrinogenemia 
E)  None of the above 
A)  Bernard-Soulier syndrome 
B)  Glanzmann's thrombasthenia 
C)  von Willebrand's disease 
D)  Congenital afibrinogenemia 
E)  None of the above

Question 11

Thrombocytosis is classified as:

Accepted Answer

A)  Platelet count 3/µL 
B)  Platelet count >400 F1F1F1S1F1F1F10 103/µL 
C)  Platelet count 100-350 F1F1F1S1F1F1F10 103/µL 
D)  Platelet count is >50 F1F1F1S1F1F1F10 103/µL 
E)  None of the above 
A)  Platelet count 3/µL 
B)  Platelet count >400 F1F1F1S1F1F1F10 103/µL 
C)  Platelet count 100-350 F1F1F1S1F1F1F10 103/µL 
D)  Platelet count is >50 F1F1F1S1F1F1F10 103/µL 
E)  None of the above

Question 12

All of the following factors are decreased in vWD except:

Accepted Answer

A)  VIII 
B)  vWF:Ag 
C)  IX 
D)  Ristocetin cofactor 
E)  von Willebrand factor (vWF) 
A)  VIII 
B)  vWF:Ag 
C)  IX 
D)  Ristocetin cofactor 
E)  von Willebrand factor (vWF)

Question 13

The P1A1 antigen is found in __________ of the normal population.

Accepted Answer

A)  97% 
B)  3% 
C)  55% 
D)  61% 
E)  35% 
A)  97% 
B)  3% 
C)  55% 
D)  61% 
E)  35%

Question 14

All of the following disorders have been associated with storage pool deficiencies except:

Accepted Answer

A)  Chédiak-Higashi syndrome 
B)  Hermansky-Pudlak syndrome 
C)  Pelger-Huët anomaly 
D)  Wiskott-Aldrich syndrome 
E)  Thrombocytopenia with absent radius (TAR) syndrome 
A)  Chédiak-Higashi syndrome 
B)  Hermansky-Pudlak syndrome 
C)  Pelger-Huët anomaly 
D)  Wiskott-Aldrich syndrome 
E)  Thrombocytopenia with absent radius (TAR) syndrome

Question 15

Uremia is a __________ platelet disorder.

Accepted Answer

A)  Congenital 
B)  Qualitative 
C)  Quantitative 
D)  Nosocomial 
E)  None of the above 
A)  Congenital 
B)  Qualitative 
C)  Quantitative 
D)  Nosocomial 
E)  None of the above

Question 16

The spleen normally pools approximately __________ of all circulating platelets.

Accepted Answer

A)  1/8 
B)  1/2 
C)  1/3 
D)  1/4 
E)  None of the above 
A)  1/8 
B)  1/2 
C)  1/3 
D)  1/4 
E)  None of the above

Question 17

What are the clinical manifestations of isoimmune-neonatal thrombocytopenia?

Accepted Answer

A)  Scattered petechiae 
B)  Platelet count below 30 F1F1F1S1F1F1F10 109/L 
C)  Purpuric hemorrhages 
D)  All of the above 
E)  None of the above 
A)  Scattered petechiae 
B)  Platelet count below 30 F1F1F1S1F1F1F10 109/L 
C)  Purpuric hemorrhages 
D)  All of the above 
E)  None of the above

Question 18

Which nonimmunologic quantitative platelet disorder is characterized by thrombocytopenia, microangiopathic hemolytic anemia, fluctuating neurologic abnormalities, renal disease, and hyaline microthrombi?

Accepted Answer

A)  Hemolytic uremic syndrome 
B)  Thrombotic thrombocytopenic purpura (TTP) 
C)  Respiratory distress syndrome 
D)  Idiopathic thrombocytopenic purpura (ITP) 
E)  None of the above 
A)  Hemolytic uremic syndrome 
B)  Thrombotic thrombocytopenic purpura (TTP) 
C)  Respiratory distress syndrome 
D)  Idiopathic thrombocytopenic purpura (ITP) 
E)  None of the above

Question 19

The prolonged bleeding time and low factor VIII levels in vWD are corrected after __________ infusion.

Accepted Answer

A)  Gamma globulin 
B)  Plasma 
C)  Platelet 
D)  Blood 
E)  None of the above 
A)  Gamma globulin 
B)  Plasma 
C)  Platelet 
D)  Blood 
E)  None of the above

Question 20

Spontaneous serious complications such as epistaxis, gingival bleeding, menorrhagia, and gastrointestinal bleeding usually are not present until the platelet count is:

Accepted Answer

A)  400-500 F1F1F1S1F1F1F10 109/L 
B)  100-400 F1F1F1S1F1F1F10 109/L 
C)  50-100 F1F1F1S1F1F1F10 109/L 
D)  9/L 
E)  None of the above 
A)  400-500 F1F1F1S1F1F1F10 109/L 
B)  100-400 F1F1F1S1F1F1F10 109/L 
C)  50-100 F1F1F1S1F1F1F10 109/L 
D)  9/L 
E)  None of the above

Exam 25: Disorders of Primary Hemostasis: Quantitative and Qualitative Platelet Disorders and Vascular Disorders

Criteria for diagnosing Glanzmann's thrombasthenia include:

Ineffective thrombopoiesis is defined as:

Platelet transfusion administration for bleeding episodes in Glanzmann's thrombasthenia is associated with:

All of the following are characteristic of hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome) except:

von Willebrand's disease (vWD) and Bernard-Soulier syndrome represent disorders of platelet __________.

The most important therapeutic intervention for suspected heparin-induced thrombocytopenia is:

__________ is a common clinical manifestation of the various paraproteinemias.

The malignant paraproteins result in:

Individuals with chronic liver disease are often associated with a significant hemorrhagic diathesis. Abnormal platelet function tests in these patients include all of the following except:

__________ is a rare autosomal recessive disorder of platelet function associated with an abnormality of membrane glycoproteins IIb and IIIa, causing decreased platelet aggregation.

Thrombocytosis is classified as:

All of the following factors are decreased in vWD except:

The P1A1 antigen is found in __________ of the normal population.

All of the following disorders have been associated with storage pool deficiencies except:

Uremia is a __________ platelet disorder.

The spleen normally pools approximately __________ of all circulating platelets.

What are the clinical manifestations of isoimmune-neonatal thrombocytopenia?

Which nonimmunologic quantitative platelet disorder is characterized by thrombocytopenia, microangiopathic hemolytic anemia, fluctuating neurologic abnormalities, renal disease, and hyaline microthrombi?

The prolonged bleeding time and low factor VIII levels in vWD are corrected after __________ infusion.

Spontaneous serious complications such as epistaxis, gingival bleeding, menorrhagia, and gastrointestinal bleeding usually are not present until the platelet count is:

Morphology of Human Blood and Marrow Cells: Hematopoiesis

Bone Marrow

The Red Blood Cell: Structure and Function

Anemia: Diagnosis and Clinical Considerations

Evaluation of Red Blood Cell Morphology and Introduction to Platelet and White Blood Cell Morphology

Iron Metabolism and Hypochromic Anemias

Megaloblastic Anemias

Aplastic Anemia Including Pure Red Cell Aplasia and Congenital Dyserythropoietic Anemia and Paroxysmal Nocturnal Hemoglobinuria

Introduction to Hemolytic Anemias: Intracorpuscular Defects: I Hereditary Defects of the Red Cell Membrane

Hemolytic Anemias: Intracorpuscular Defects: II Hereditary Enzyme Deficiencies

Hemolytic Anemias: Intracorpuscular Defects: III the Hemoglobinopathies

Hemolytic Anemias: Intracorpuscular Defects: Iv Thalassemia

Hemolytic Anemias: Extracorpuscular Defects

Hypoproliferative Anemia: Anemia Associated With Systemic Diseases

Cell Biology, Disorders of Neutrophils, Infectious Mononucleosis, and Reactive Lymphocytosis

Introduction to Leukemia and the Acute Leukemias

Chronic Myeloproliferative Disorders I: Chronic Myelogenous Leukemia

Chronic Myeloproliferative Disorders Ii: Polycythemia Vera, Essential Thrombocythemia, and Idiopathic Myelofibrosis

Myelodysplastic Syndromes

Chronic Lymphocytic Leukemia and Related Lymphoproliferative Disorders

The Lymphomas

Multiple Myeloma and Related Plasma Cell Disorders

Lipid Lysosomal Storage Diseases and Histiocytosis

Introduction to Hemostasis

Disorders of Plasma Clotting Factors

Interaction of the Fibrinolytic, Coagulation, and Kinin Systems; Disseminated Intravascular Coagulation; and Related Pathology

Introduction to Thrombosis and Anticoagulant Therapy

Quality Control in the Hematology Laboratory

Body Fluid Examination: the Qualitative, Quantitative, and Morphologic Analysis of Serous, Cerebrospinal, and Synovial Fluids

Hematology Methods

Principles of Automated Differential Analysis

Coagulation Procedures

Applications of Flow Cytometry to Hematology and Hemostasis

Molecular Diagnostic Techniques in Hematopathology

Special Stainscytochemistry

Filters

The P1^A1antigen is found in __________ of the normal population.