Platelet disorders+miscellaneous Uworld key factss

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100 Questions

Question 1: What is the primary function of von Willebrand factor (vWF) in hemostasis?

Mediation of platelet adhesion to subendothelial collagen

Question 2: In vWF deficiency, which laboratory test measures vWF-dependent platelet agglutination?

Ristocetin cofactor assay

Question 3: How does the ristocetin cofactor assay work in diagnosing vWF deficiency?

It evaluates platelet aggregation via vWF-GP Ib receptor interaction.

Question 5: Which bleeding disorder is characterized by thrombocytopenia, enlarged platelets, and mucocutaneous bleeding due to a deficiency of glycoprotein Ib (GP Ib) receptors?

Bernard-Soulier syndrome

Question 6: How does Glanzmann thrombasthenia differ from Bernard-Soulier syndrome in terms of platelet agglutination response to ristocetin?

Glanzmann thrombasthenia shows normal agglutination, while Bernard-Soulier syndrome shows abnormal agglutination.

Question 7: What is the primary function of von Willebrand factor (vWF) in hemostasis?

Mediation of platelet adhesion to subendothelial collagen

Question 7: Which bleeding disorder is characterized by mucocutaneous bleeding, normal platelet agglutination response to ristocetin, and deficiency of glycoprotein IIb/IIIa receptors.

b) Glanzmann thrombasthenia

Question 8: What is the role of von Willebrand factor (vWF) in relation to factor VIII.

c) vWF carries and stabilizes factor VIII, prolonging its half-life.

Question 9: Which treatment can be used for menorrhagia in patients with vWF deficiency.

d) Combined oral contraceptives

Question 10: What is the primary effect of thromboxane A2 deficiency due to aspirin treatment.

a) Impaired platelet aggregation

Question 11: In congenital deficiency of factor XII (Hageman), what is the characteristic laboratory finding.

b) Prolonged activated partial thromboplastin time (aPTT)

Question 13: What is the primary function of ristocetin in the ristocetin cofactor assay.

a) To activate GP Ib receptors

Question 14: Which component is responsible for platelet adhesion to subendothelial collagen after endothelial damage.

a) von Willebrand factor (vWF)

Question 15: Which statement accurately describes the role of vWF in platelet adhesion.

d) vWF mediates platelet adhesion by binding to GP Ib receptors.

Question 1: What is the primary reason for impaired generation of new blood cells in response to acute stressors such as blood loss or hypoxia in older individuals?

Decreased bone marrow mass & Decreased response to stimulatory cytokines

Question 2: Which of the following changes in the bone marrow composition is typically observed in older patients?

Reduced overall mass

Question 3: In comparison to younger patients, how does the bone marrow of older patients generally respond to stimulatory cytokines?

Impaired response

Question 4: What is the significance of increased bone marrow fat in older individuals?

It contributes to the reduced functional reserve of hematopoietic cells

Question 5: Which factor limits the capacity of older individuals to rapidly generate new blood cells?

Impaired bone marrow reserve

Question 8: What is the primary cause of increased medullary cavity size in patients with advanced age?

b) Loss of endosteal cortical bone

Question 9: Which condition is characterized by anemia, extramedullary hematopoiesis (hepatosplenomegaly), and bone marrow fibrosis?

b) Myelofibrosis

Question 10: In older individuals, what is the consequence of having a reduced overall bone marrow mass?

c) Impaired functional reserve of hematopoietic cells

Question 11: What is the primary cause of increased medullary cavity size in patients with advanced age?

b) Loss of endosteal cortical bone

Which cytokine stimulates the liver to increase production of thrombopoietin in infections?

Interleukin-6

What term is used to describe the subsequent increase in serum platelet count in response to elevated thrombopoietin levels during infections?

Reactive thrombocytosis

Which condition is most likely to cause elevated platelet count due to the release of inflammatory cytokines?

Reactive thrombocytosis

What is the role of hepcidin in the context of elevated inflammatory cytokine levels?

It down-regulates iron absorption and iron release.

Which term is used to describe the anemia characterized by reduced iron availability for reticulocytosis due to inflammatory cytokine-induced hepcidin release?

Microcytic anemia

Question 7: What is the primary cause of reactive thrombocytosis in the setting of infections?

High levels of interleukin-6 (IL-6)

Question 8: Which condition leads to increased erythrocytosis?

Chronic hypoxemia

Question 9: Which of the following terms describes a state of elevated platelet count due to ongoing infection or inflammation?

Reactive thrombocytosis

Question 10: What is the most common cause of elevated platelet count in various age groups?

Reactive thrombocytosis

Question 11: What is the primary cause of essential thrombocythemia?

None

Question 1: What is the likely cause of exertional dyspnea, fatigue, conjunctival pallor, macrocytosis, and hypersegmented neutrophils?

Vitamin B12 deficiency

Question 2: Which vitamin acts as a cofactor in the recycling of 5-methyl-tetrahydrofolate to tetrahydrofolate?

Vitamin B12 (cobalamin)

Question 3: Why are hematopoietic cells primarily affected in megaloblastic anemia due to vitamin B12 deficiency?

Their low replication rate

Question 4: What is the primary impact of folate or vitamin B12 deficiency on DNA synthesis and cell cycle progression?

Impaired S-phase progression

Question 5: What term describes the condition in which nuclear division slows relative to cytoplasmic maturation?

Megaloblastic anemia

Question 6: Which of the following complications is more likely to be associated with vitamin B12 deficiency rather than folate deficiency?

Symmetric paresthesia

Question 7: What term is used to describe the condition marked by the destruction of hematopoietic stem cells and replacement of bone marrow with fat cells?

Aplastic anemia

Question 8: What would be a distinguishing feature on peripheral blood smear in megaloblastic anemia due to vitamin B12 deficiency?

Nucleated red blood cells

Question 9: Which laboratory finding is characteristic of chronic kidney disease, rather than megaloblastic anemia?

Normocytic anemia

Question 10: What condition typically causes traumatic hemolysis due to red blood cell shearing, resulting in schistocytes on peripheral blood smear?

Aortic stenosis

Which one of these is the major form of hemoglobin in adults?

2 alpha and 2 beta chains

Which protein is primarily responsible for oxygen storage in skeletal and cardiac muscle tissue?

Myoglobin

What shape is the oxygen-hemoglobin dissociation curve for myoglobin?

Hyperbolic

Question 7: What is the main difference between the oxygen-hemoglobin dissociation curve of hemoglobin A and that of individual hemoglobin subunits?

Individual subunits have a hyperbolic curve, while hemoglobin A has a sigmoidal curve

Question 8: Which factor would cause the oxygen-hemoglobin dissociation curve to shift to the right.

A low oxygen affinity hemoglobin

Question 9: A left shift in the oxygen-hemoglobin dissociation curve indicates:

A high oxygen affinity hemoglobin

Question 10: Which one of these is the most correct?

They share a similar primary structure but have different secondary and tertiary structures

Question 11: Which one of these is the most correct about hemoglobin and myoglobin?

They have almost identical secondary and tertiary structures

Which enzyme is primarily inhibited by aspirin when administered in low doses, resulting in a sustained decrease in thromboxane A2 production and subsequent antithrombotic effects?

COX-1

What is the mechanism by which aspirin achieves its prolonged antithrombotic effect when administered in low doses?

Irreversible acetylation and inhibition of COX-1

What is the main product of COX-2 enzyme activity, which is inducibly expressed and contributes to fever, inflammation, and pain sensitivity?

Prostaglandins

Aspirin inhibits COX-1 by:

Irreversible acetylation

Why is aspirin unique compared to other NSAIDs in terms of its effect on platelet function?

Reducing platelet aggregation

Which of the following is a primary effect of aspirin's inhibition of COX-2?

Decreased prostaglandin production

What is the primary reason for aspirin's antithrombotic effect to persist even after the drug is discontinued?

Aspirin delays the release of thromboxane A2 from platelets

Why is it recommended to discontinue aspirin at least 5 days prior to surgery?

To avoid excessive bleeding due to prolonged platelet inhibition

Which enzyme is gets irreversible acetylation by aspirin?

COX-1

Why can other NSAIDs be discontinued immediately before surgery, unlike aspirin?

They do not affect platelet function

Which of the following is the role of the flanking sequences adjacent to the target region in PCR?

They serve as primers to start DNA replication.

Which step of PCR involves thermal separation of the DNA template into single strands?

Denaturing

What is the purpose of the annealing step in PCR?

To combine primers with the denatured, single-stranded flanking ends

Which enzyme is responsible for forming new daughter DNA strands in the 5' to 3' direction during the elongation step of PCR?

DNA polymerase

What is the primary goal of PCR?

To amplify specific DNA segments, even without knowing their exact sequence

Which technique is commonly used to separate DNA fragments based on size?

E) Gel electrophoresis

Which enzyme is responsible for the subsequent catabolism of 2,3-BPG to 3-phosphoglycerate in erythrocytes?

Bisphosphoglycerate phosphatase

What effect does higher 2,3-BPG levels have on oxygen delivery to the tissues by hemoglobin?

It increases oxygen delivery to the tissues

How does the conversion of 1,3-BPG to 2,3-BPG affect the net ATP gain in erythrocytes?

It decreases net ATP production

What is the primary function of von Willebrand factor (vWF) in hemostasis?

It stabilizes factor VIII in the blood

Which enzyme is primarily inhibited by aspirin when administered in low doses, resulting in a sustained decrease in thromboxane A2 production and subsequent antithrombotic effects?

Cyclooxygenase-1 (COX-1)

Which of the following is the likely consequence of a β-globin mutation that decreases the positive charge of the 2,3-BPG binding pocket in hemoglobin?

Increased affinity of hemoglobin for oxygen

What is the primary function of 2,3-BPG in relation to hemoglobin?

Decrease oxygen-hemoglobin affinity

How does fetal hemoglobin (HbF) differ from adult hemoglobin A in terms of its binding affinity to 2,3-BPG?

HbF does not bind to 2,3-BPG as well as HbA.

What is the role of the γ chains in fetal hemoglobin (HbF) in terms of 2,3-BPG binding?

They increase HbF's oxygen affinity.

Question 7: What effect does a decrease in positive charge within the 2,3-BPG binding pocket of hemoglobin have on its interaction with 2,3-BPG?

It weakens the interaction.

Question 8: Which type of mutation in the β-globin chain leads to the formation of hemoglobin C?

Replacement of a glutamate by lysine

Question 9: How does hemoglobin C affect the affinity of hemoglobin for oxygen and 2,3-BPG?

It has no effect on affinity for oxygen or 2,3-BPG.

Question 10: What characteristic of fetal hemoglobin (HbF) is responsible for its higher oxygen affinity compared to adult hemoglobin A?

Replacement of histidine with serine in γ chains leading to reduction of the positive charge pocket in the hemoglobin protein.

Question 11: What effect does a decrease in positive charge within the 2,3-BPG binding pocket of hemoglobin have on its interaction with 2,3-BPG?

It weakens the interaction.

Question 8: What is the primary role of ADAMTS13 deficiency in the development of thrombotic thrombocytopenic purpura (TTP)?

Promoting platelet aggregation

Question 9: Which statement accurately describes the diagnostic criteria of TTP?

Thrombocytopenia and microangiopathic hemolytic anemia are essential for diagnosis.

Which epidermal growth factor receptor is commonly overexpressed by aggressive breast cancers and targeted by monoclonal antibodies like trastuzumab?

HER2

What is the primary mechanism of tumor regression seen with monoclonal antibody treatment targeting HER2 in breast cancer?

Antibody-dependent cellular cytotoxicity (ADCC)

Which step of ADCC involves the release of granules containing perforin and granzymes by natural killer (NK) cells?

NK cell binding to target cell Fc portion

What is the role of CD16 in antibody-dependent cellular cytotoxicity (ADCC)?

It facilitates NK cell attachment to antibody-bound cells

What is the primary reason natural killer (NK) cells are considered part of the innate immune system?

They do not interact with MHC complexes to differentiate self from nonself

What distinguishes natural killer (NK) cells from cytotoxic T-cells in their recognition of tumor cells?

NK cells target tumor cells expressing MHC class I

Which one of these is the most correct about hemoglobin and myoglobin?

Myoglobin has a higher affinity for oxygen than hemoglobin

Which enzyme is responsible for forming new daughter DNA strands in the 5' to 3' direction during the elongation step of PCR?

DNA polymerase III

What is the main product of COX-2 enzyme activity, which is inducibly expressed and contributes to fever, inflammation, and pain sensitivity?

Prostaglandin E2

Question 1: In which cellular organelles does the first and the final 3 steps of heme synthesis occur?

Mitochondria

Question 2: What is the primary site of heme synthesis in the body?

Bone marrow

Question 3: What is the lifespan of mature red blood cells (erythrocytes) in circulation?

120 days

Question 4: Which type of cells principally synthesize heme in the bone marrow?

Erythrocyte precursor cells

Question 5: What is the fate of erythrocyte precursor cells as they mature into mature red blood cells?

They lose their nuclei and mitochondria

Question 6: What is the significance of erythrocytes losing their mitochondria during maturation?

They lose the ability to generate heme and hemoglobin

Question 7: Which cellular organelles typically disappear during erythrocyte development?

Nucleus

Question 10: What is the primary reason for impaired generation of new blood cells in response to acute stressors such as blood loss or hypoxia in older individuals?

Decreased hematopoietic stem cell function

Test your knowledge of von Willebrand factor (vWF) and platelet disorders with this quiz. Explore the role of vWF in hemostasis, laboratory tests for vWF deficiency, and different platelet disorders such as Glanzmann thrombasthenia and Bernard-Soulier syndrome.

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