2-Hematologic
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Questions and Answers

What is the primary action of hemostasis following vessel injury?

  • To enhance blood flow through the injured vessel
  • To promote rapid clot formation
  • To limit blood loss from an injured vessel (correct)
  • To prevent vascular constriction

Which of the following factors is not a potent vasoconstrictor involved in vasoconstriction after vessel injury?

  • Fibrinopeptides
  • Thromboxane A2 (TXA2)
  • Endothelin
  • Nitric oxide (correct)

What is the effect of an imbalance in hemostasis?

  • Increased recovery of injured tissue
  • Consistent blood flow restoration
  • Severe blood loss or excessive clot formation (correct)
  • Stabilization of vascular health

Which combination of substances is involved in the vasoconstriction process after vessel injury?

<p>TXA2, Endothelin, Serotonin, and Bradykinin (D)</p> Signup and view all the answers

What is the role of platelets in the hemostatic process?

<p>To interact with the endothelium and coagulation factors (D)</p> Signup and view all the answers

Which drug is known to irreversibly inhibit platelet function?

<p>Prasugrel (C)</p> Signup and view all the answers

What is a potential consequence of low fibrinogen levels?

<p>Acquired hypofibrinogenic state (C)</p> Signup and view all the answers

Which of the following conditions may develop features of Thrombotic Thrombocytopenic Purpura (TTP)?

<p>HIV (C)</p> Signup and view all the answers

How does portal hypertension relate to blood platelets?

<p>Increases platelet sequestering in the spleen (B)</p> Signup and view all the answers

What is a treatment method recommended to maintain adequate perfusion?

<p>Plasmapheresis (B)</p> Signup and view all the answers

In qualitative disorders of hemostasis, what is often impaired alongside thrombocytopenia?

<p>Platelet function (B)</p> Signup and view all the answers

Which factor is essential to replace during treatment for hemostatic disorders?

<p>Fibrinogen concentrate (A)</p> Signup and view all the answers

What condition is characterized by high blood viscosity and increased platelet count?

<p>Myeloproliferative disease (B)</p> Signup and view all the answers

Which statement accurately describes the relationship between heart rate and volume resuscitation?

<p>A heart rate of 120 beats per minute indicates 1 ml/kg/hr fluid resuscitation. (A)</p> Signup and view all the answers

What is one of the key principles of damage control resuscitation?

<p>Utilize permissive hypotension in managing hypotensive patients. (D)</p> Signup and view all the answers

Which factor is associated with severe bleeding diathesis?

<p>Factor XIII deficiency. (B)</p> Signup and view all the answers

What is the primary mediator of platelet aggregation?

<p>Thromboxane A2. (B)</p> Signup and view all the answers

Which statement is true about the male to female ratio in certain conditions?

<p>It is 1:1. (B)</p> Signup and view all the answers

In which condition is bleeding typically delayed?

<p>Factor XIII deficiency. (D)</p> Signup and view all the answers

Which enzyme is primarily responsible for clot degradation?

<p>Plasmin. (D)</p> Signup and view all the answers

What role does thromboxane A2 play in haemostasis?

<p>It promotes vasoconstriction and platelet aggregation. (C)</p> Signup and view all the answers

Which statement accurately describes the relationship between thrombocytopenic purpura and impaired platelet function?

<p>Thrombotic thrombocytopenic purpura is linked to the activation of the immune response. (D)</p> Signup and view all the answers

Identify the statement that correctly represents what factor XII activation signifies in the intrinsic coagulation pathway.

<p>It is initiated following injury to the subendothelium. (B)</p> Signup and view all the answers

Which of the following is a required component for effective platelet adhesion during primary hemostasis?

<p>von Willebrand factor (C)</p> Signup and view all the answers

What is indicated by an elevated activated partial thromboplastin time (aPTT) in patients?

<p>Intrinsic pathway dysfunction (C)</p> Signup and view all the answers

Which substances are known as the primary mediators of platelet aggregation?

<p>ADP and Serotonin (C)</p> Signup and view all the answers

What does the term 'idiopathic' in idiopathic thrombocytopenic purpura refer to?

<p>Unidentified cause of low platelet count (D)</p> Signup and view all the answers

Which of the following describes a characteristic of vasoconstriction in primary hemostasis?

<p>It is pronounced in vessels with smooth muscle. (A)</p> Signup and view all the answers

What is the primary role of platelets during the initial phase of hemostasis?

<p>To aggregate at the injury site and form an initial platelet plug (A)</p> Signup and view all the answers

Which protein is responsible for binding platelets to the subendothelial collagen during primary hemostasis?

<p>Von Willebrand factor (vWf) (B)</p> Signup and view all the answers

What occurs to a platelet's shape upon activation?

<p>It transforms into a spherical shape (D)</p> Signup and view all the answers

What is the subsequent phase that follows primary hemostasis?

<p>Coagulation phase (D)</p> Signup and view all the answers

Why is it important for clots to be removed during the healing process?

<p>To prevent excessive clotting and potential thrombus formation (B)</p> Signup and view all the answers

What triggers the activation of platelets at the site of injury?

<p>Interaction with subendothelial tissue components (D)</p> Signup and view all the answers

What is a characteristic feature of platelets in their inactive state?

<p>They appear discoid (C)</p> Signup and view all the answers

During the hemostatic process, what role does thrombin play?

<p>It aids in the transformation of fibrinogen to fibrin (D)</p> Signup and view all the answers

What is the first line of treatment for Idiopathic Thrombocytopenic Purpura (ITP)?

<p>Corticosteroids (A)</p> Signup and view all the answers

Which disorder is characterized by a defect in the GP IIb/IIIa complex?

<p>Glanzmann Thrombasthenia (C)</p> Signup and view all the answers

What triggers a drop in platelet count in Heparin Induced Thrombocytopenia (HIT)?

<p>Heparin administration (B)</p> Signup and view all the answers

Which of the following conditions is associated with the presence of schistocytes?

<p>Thrombotic Thrombocytopenic Purpura (TTP) (B)</p> Signup and view all the answers

Which secondary condition is most likely to cause Hemolytic Uremic Syndrome (HUS)?

<p>Escherichia coli O157:H7 (C)</p> Signup and view all the answers

What is the primary characteristic of Thrombotic Thrombocytopenic Purpura (TTP)?

<p>Large vWF molecules interaction with platelets (B)</p> Signup and view all the answers

What treatment is considered a third-line therapy for ITP?

<p>Thrombopoietin receptor agonist (D)</p> Signup and view all the answers

What is a common feature of Storage Pool Disease?

<p>Loss of dense granules (A)</p> Signup and view all the answers

Which mechanism describes the decreased survival of platelets in conditions like ITP?

<p>Destruction of platelets (D)</p> Signup and view all the answers

What can potentially lead to thrombocytopenia and intravascular thrombosis in patients?

<p>Hematologic malignancies (C)</p> Signup and view all the answers

What does a positive ELISA test indicate in the context of Heparin Induced Thrombocytopenia (HIT)?

<p>Confirmatory for HIT (D)</p> Signup and view all the answers

Which treatment option is recommended to manage HIT?

<p>Stop heparin and switch to an alternative anticoagulant (B)</p> Signup and view all the answers

Which condition primarily arises in children after a viral illness?

<p>Acute ITP (B)</p> Signup and view all the answers

In the context of autoimmune disorders, what is a significant consequence of platelet destruction?

<p>Thrombocytopenia (A)</p> Signup and view all the answers

Flashcards

Hemostasis

The process that prevents blood loss from a damaged blood vessel, localizes the injury, repairs the vessel, and re-establishes blood flow.

Endothelium

A type of specialized tissue that lines the inside of blood vessels and plays a key role in blood clotting.

Platelets

tiny blood cells that help stop bleeding by forming a plug at the site of injury.

Coagulation factors

Chemical messengers involved in blood clotting and other bodily functions.

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Thromboxane A2 (TXA2)

A powerful vasoconstrictor produced by platelets that helps narrow blood vessels, reducing blood flow to the injured area.

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Primary Hemostasis

The initial step in hemostasis, where platelets form a temporary plug at the site of injury.

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von Willebrand Factor (vWf)

A protein that helps platelets stick to the subendothelium and to each other.

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Platelet Activation

The process by which platelets change shape from disc-like to spherical, releasing granules and becoming activated.

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Platelet Granules

These are released by activated platelets and help with clotting and inflammation.

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Initial Platelet Plug

The temporary plug formed by platelets at the site of injury during primary hemostasis.

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Coagulation Phase

The process of forming a stable clot at the site of injury, involving a cascade of reactions involving clotting factors. It follows primary hemostasis.

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Fibrinolysis

The final phase of hemostasis, where the clot is gradually dissolved by enzymes. This ensures the blood vessel is cleared and function is restored.

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Intrinsic Pathway

The intrinsic pathway is activated when there's damage to blood vessel walls, leading to the formation of a blood clot.

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Elevated aPTT

Elevated aPTT (activated partial thromboplastin time) is a hallmark of an abnormal intrinsic pathway function.

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Primary Hemostasis: Vasoconstriction

The first phase of primary hemostasis involves vasoconstriction, which helps reduce blood loss by narrowing blood vessels.

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Platelet Adhesion

von Willebrand factor (vWF) is crucial for platelet adhesion, the process of platelets sticking to the damaged vessel wall.

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Primary Hemostasis: Platelet Aggregation

The second phase of primary hemostasis involves platelet aggregation, where platelets clump together at the site of injury.

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Platelet Aggregation Mediators

ADP (adenosine diphosphate) and serotonin are major players in platelet aggregation.

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Coagulation Cascade

The coagulation cascade is a series of events that lead to the formation of a stable fibrin clot.

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TTP

Thrombotic thrombocytopenic purpura (TTP) is a rare but serious condition characterized by platelet consumption and the formation of microthrombi (small blood clots) in small blood vessels.

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Factor XIII

Factor XIII is a clotting factor associated with severe bleeding diathesis, with a 1:1 male to female ratio. It is a delayed bleeding disorder.

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Clot Degradation Enzyme

Plasmin is responsible for breaking down clots. It is involved in clot degradation.

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Damage Control Resuscitation

Damage control resuscitation emphasizes minimizing crystalloid-based resuscitation. It involves immediate release and administration of pre-defined blood products, often with permissive hypotension.

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Thromboxane A2

Thromboxane A2 contributes to both platelet aggregation and vasoconstriction. It plays a key role in hemostasis.

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Vasoconstriction Mediators

Bradykinin, fibrinopeptides, and endothelin are involved in vasoconstriction. They play a crucial role in regulating hemostasis.

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Thrombin

Thrombin is a key enzyme involved in the coagulation cascade. It plays a vital role in converting fibrinogen to fibrin, which forms the meshwork of a clot.

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Hypofibrinogenemia

A condition where there is a deficiency in fibrinogen, a key clotting factor in the blood.

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Myeloproliferative Disorders

This is a group of conditions that increase the production of blood cells in the bone marrow, including red blood cells, white blood cells, and platelets. The platelet count can be very high in such cases.

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Polycythemia Vera

A type of myeloproliferative disorder characterized by an overproduction of red blood cells, leading to an increase in blood viscosity (thickness) which can cause blood clots.

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Primary Fibrinolysis

The process of fibrinolysis (breakdown of blood clots), where the enzyme urokinase breaks down fibrin, the main component of blood clots.

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Antifibrinolytic Agents

Medicines that prevent the breakdown of clots by inhibiting the activity of fibrinolytic enzymes.

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Thrombocytopenia

This refers to a decrease in the number of platelets, which are crucial for blood clotting.

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Thrombocytosis

The condition in which the formation of blood clots is increased, often due to certain drugs and disease states.

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GP IIb/IIIa Inhibitors

A group of drugs that inhibit platelet function by irreversibly blocking the platelet receptor GP IIb/IIIa, crucial for platelet activation and aggregation.

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Glanzmann Thrombasthenia

A rare, inherited bleeding disorder caused by a deficiency or dysfunction of the GP IIb/IIIa complex, which is crucial for platelet aggregation. This results in impaired platelet adhesion and increased bleeding.

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Bernard-Soulier Syndrome

A rare, inherited bleeding disorder caused by a defect in the GP Ib/IX/V receptor, which is responsible for platelet adhesion to von Willebrand factor (vWF). This leads to impaired platelet adhesion and increased bleeding.

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Storage Pool Disease

A disorder characterized by a deficiency in dense granules, which are storage sites for ADP, ATP, Ca, and inorganic phosphates within platelets. This leads to impaired platelet aggregation and prolonged bleeding time.

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Acute Idiopathic Thrombocytopenic Purpura (ITP)

A condition caused by the destruction of platelets in the body, often associated with a viral illness in children. It leads to a low platelet count (thrombocytopenia) and increased bleeding.

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Chronic Idiopathic Thrombocytopenic Purpura (ITP)

A chronic form of ITP with no identifiable cause, typically affecting adults. It results from impaired platelet production and a T-cell mediated autoimmune response.

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Disseminated Intravascular Coagulation (DIC)

A rare, life-threatening condition characterized by widespread activation of platelets and clotting within blood vessels. It leads to severe bleeding and clotting abnormalities.

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Heparin Induced Thrombocytopenia (HIT)

A type of immune-mediated thrombocytopenia that can occur after exposure to heparin, a medication used to prevent blood clots. It causes both low platelet counts and a risk of thrombosis.

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Thrombotic Thrombocytopenic Purpura (TTP)

A serious condition involving platelet activation and formation of microthrombi (small blood clots) within blood vessels. It affects the microcirculation and can lead to organ damage, particularly in the kidneys and brain.

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Hemolytic Uremic Syndrome (HUS)

A condition similar to TTP, but often triggered by E. coli O157:H7 infection. It also involves microthrombi, leading to hemolytic anemia, kidney failure, and sometimes neurological symptoms.

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Platelet Production Deficiency

A condition caused by a deficiency in the production of platelets. It is often associated with leukemia, myeloproliferative disorders, nutritional deficiencies (e.g., B12 or Folate), or exposure to certain drugs like chemotherapy or radiation.

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Decreased Platelet Survival

A condition where the lifespan of platelets is shortened, leading to decreased platelet counts. This can be caused by various factors such as autoimmune disorders, infections, or medications.

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Platelet Adhesion Defect

A condition where platelets cannot properly attach to damaged blood vessels, leading to prolonged bleeding. This often involves malfunctions in specific surface proteins.

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Immune-Mediated Thrombocytopenia

A condition characterized by the destruction of platelets by the body's own immune system. This can occur in various forms, including ITP and HIT.

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Platelet Thrombi

A condition where platelets are clumped together within blood vessels, leading to clotting and potential complications like organ damage.

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Large vWF Molecules

A condition that involves the abnormal production of very large molecules of von Willebrand factor (vWF), which are essential for platelet adhesion. These large vWFs can cause platelet clumping and can be a risk for blood clotting disorders.

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Study Notes

Hemostasis

  • Hemostasis is a process that stops bleeding
  • It involves vascular constriction, platelet plug formation, fibrin clot formation, and fibrinolysis
  • The four processes interrelate to limit blood loss

Vascular Constriction

  • Initial response to vessel injury
  • More pronounced in vessels with medial smooth muscle walls

Primary Hemostasis

  • Platelet plug formation
  • Involves platelet adhesion, activation, and aggregation

Platelet Function

  • Anucleate fragments of megakaryocytes
  • 150,000-400,000/µL (normal value)
  • 30% sequestered in the spleen
  • Average lifespan: 7-10 days
  • Play a role in forming hemostatic plugs and thrombin formation
  • Do not normally adhere to each other or to the vessel wall

Secondary Hemostasis

  • Fibrin clot formation
  • Involves a cascade of activation of coagulation factors
  • The final step in clot formation is fibrin polymerization

Fibrinolysis

  • Clot destruction
  • Necessary for preventing or resolving unwanted blood clots
  • Initiated by tissue plasminogen activator (tPA)

Coagulation Factors

  • Enzymes that regulate the clotting cascade
  • Zymogens, non-enzymatic cofactors, and calcium
  • Mostly produced in the liver and circulate in inactive precursor form

Coagulation Factor Deficiencies

  • Factor VIII deficiency (Hemophilia A)
  • Factor IX deficiency (Hemophilia B)
  • Factor XI deficiency (Hemophilia C)
  • Factor V Leiden
  • Factor VII deficiency
  • Factor XIII deficiency
  • Von Willebrand's disease

Platelet Functional Defects

  • Glanzmann Thrombasthenia
  • Bernard-Soulier Syndrome
  • Storage pool disease
  • Decreased survival
  • Failure of production

Acquired Coagulation Inhibitors

  • Antiphospholipid syndromes (APLS)
  • Lupus anticoagulant and anticardiolipin antibodies

Complications of Transfusion

  • Blood-induced pro-inflammatory responses
  • Transfusion-related acute lung injury (TRALI)
  • Acute hemolytic transfusion reaction
  • Febrile nonhemolytic reactions
  • Transfusion-associated circulatory overload (TACO)
  • Bacterial contamination

Transfusion

  • Autologous transfusion: Patients can donate blood
  • Banked whole blood: Shelf life is 42 days
  • Frozen red blood cells: Shelf life is 10 years
  • Leukocyte-reduced/washed RBCs: Prevention of non-hemolytic transfusion reactions
  • Fresh frozen plasma: Shelf life is 2 years
  • Platelet concentrates: For thrombocytopenia and massive blood loss; shelf life is 120 hours

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Test your knowledge on the processes involved in hemostasis and the role of platelets in vascular injury response. This quiz covers factors affecting vasoconstriction, treatments for hemostatic disorders, and complications arising from imbalances in hemostasis. Dive into the intricate mechanisms that regulate blood clotting and vessel health.

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