Pharmacotherapy of Coagulation Disorders

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Questions and Answers

Which subtype of Type 2 VWF disease causes mucocutaneous bleeding due to low VWF in the blood?

Type 2M

Type 2A (correct)

Type 2B

Type 2D

What genetic pattern is associated with Type 3 VWF disease?

X-linked Dominant

Autosomal Recessive (correct)

X-linked Recessive

Autosomal Dominant

Which laboratory test is most relevant for diagnosing Type 2B VWF disease?

Factor VIII clotting test

CBC (correct)

Ristocetin co-factor test

VWF antigen test

Which treatment is contraindicated for patients with Type 2B VWF disease?

Desmopressin Signup and view all the answers

Which of the following statements about Pseudo Willebrand disease is true?

It is associated with prolonged bleeding. Signup and view all the answers

What role does Factor VI play in blood coagulation?

It has no role in blood coagulation. Signup and view all the answers

Which drug is classified as a Vitamin K Epoxide reductase inhibitor?

Warfarin Signup and view all the answers

What is the primary issue faced by individuals with Hemophilia?

Severe reduction in blood clotting ability. Signup and view all the answers

What is the main function of Hemostasis in the body?

It maintains blood fluidity and prevents excessive bleeding. Signup and view all the answers

Which drug is identified as an ADP inhibitor?

Clopidogrel Signup and view all the answers

What is the primary treatment for a warfarin overdose?

Vitamin K Signup and view all the answers

Which laboratory tests are essential for diagnosing clotting factor VII deficiency?

APTT, PT, and platelet count Signup and view all the answers

What disorder is characterized by a deficiency in Factor V and has less than 200 reported cases worldwide?

Owren's disease Signup and view all the answers

What is a common complication associated with Hageman factor deficiency?

Increased risk for DVT Signup and view all the answers

Which factor deficiency is indicated by prolonged PT but normal APTT?

Clotting Factor VII deficiency Signup and view all the answers

Which test assesses the effectiveness of vitamin K in the body?

Prothrombin time (PT) Signup and view all the answers

Which of the following symptoms is NOT associated with clotting factor deficiency?

Persistent cough Signup and view all the answers

What treatment is indicated for congenital Clotting Factor X deficiency?

Factor X replacement therapy Signup and view all the answers

What is primarily affected in Hemophilia A?

Clotting factor VIII Signup and view all the answers

Which of the following is NOT a symptom of hemophilia?

Hypercoagulation Signup and view all the answers

Which treatment is C/I for patients with hematuria?

Anti-fibrinolytics Signup and view all the answers

What distinguishes acquired hemophilia from inherited hemophilia?

It is caused by autoantibodies against clotting factors. Signup and view all the answers

What is the most likely bleeding site in Hemophilia B?

Joints Signup and view all the answers

Which assay is specifically utilized for diagnosing Hemophilia B?

FIX assay Signup and view all the answers

What is the mechanism of action of Desmopressin?

Induces cAMP activation Signup and view all the answers

What type of disorder is Hemophilia A classified as?

X-linked recessive Signup and view all the answers

Which of the following can be a treatment for inherited Hemophilia?

Factor IX infusion Signup and view all the answers

Which factor deficiency is associated with prothrombin?

Factor II Signup and view all the answers

Study Notes

Coagulation Disorders Overview

Hemostasis maintains blood fluidity, repairs vascular injury, limits blood loss, and prevents thrombosis.
Understanding the coagulation cascade and its components is essential for diagnosing and treating disorders.

Clotting Factors & Their Role

Factor VI is not involved in blood coagulation.
Clotting factors are crucial in the coagulation cascade, each fulfilling a specific function.

Anticoagulant Drugs

Aspirin inhibits thromboxane A2 to prevent platelet aggregation.
Clopidogrel acts as an ADP inhibitor to reduce thrombus formation.
Abciximab is a GP IIb/IIIa receptor inhibitor.
Warfarin inhibits Vitamin K Epoxide reductase affecting factors II, VII, IX, and X.
Heparin and Dalteparin activate Antithrombin III.
Dabigatran inhibits thrombin (Factor IIa).
Rivaroxaban targets Factor Xa.
Alteplase functions as a Tissue Plasminogen Activator for clot lysis.

Hemophilia Types

Hemophilia is known as "Royal Disease" due to its prevalence among royals; characterized by severe bleeding.
Hemophilia A is due to factor VIII deficiency, 4 times more common than Hemophilia B (factor IX deficiency).
Incidence of hemophilia is approximately 1 in 5000 male births.
Symptoms include easy bruising and spontaneous hemorrhage after injuries.

Hemophilia A Specifics

X-linked recessive disorder; traced to defective factor VIII.
Symptoms: Anemia, weakness, tachycardia, and various types of hemorrhage, including hematuria.
Diagnosis involves CBC, PT, aPTT, and specific factor VIII assays.
Treatment focuses on managing bleeding episodes, factor replacement, and anti-fibrinolytics.

Desmopressin

Analog of vasopressin, acts mostly through V2 receptors.
Induces exocytosis of von Willebrand Factor (VWF), enhancing clotting function.

Acquired Hemophilia

Rare, life-threatening condition with autoantibodies against factor VIII.
Symptoms include diverse bleeding manifestations; diagnosis shows prolonged aPTT and decreased factor VIII.
Treatment combines factor VIII infusion and immunosuppression, using corticosteroids and monoclonal antibodies like Rituximab.

Hemophilia B Specifics

Caused by factor IX deficiency; referred to as "Christmas disease."
Symptoms primarily involve joint bleeding (hemarthrosis) and hematomas.
Diagnosis includes platelet count and specific assays for factor IX.
Treatment involves factor IX replacement and anti-fibrinolytics.

Clotting Factor Deficiencies

Factor II deficiency: Leads to hypoprothrombinemia; can be inherited, autoimmune, or drug-induced. Symptoms may include severe bleeding.
Factor V deficiency (Owren’s disease): Extremely rare, characterized by prolonged PT and aPTT; treatment involves fresh frozen plasma.
Factor VII deficiency: Prolonged PT with normal aPTT; management includes specific factor replacement and anti-fibrinolytics.
Factor X deficiency: Related to vitamin K metabolism; shows prolonged PT and aPTT; treatment involves vitamin K and factor replacement.

von Willebrand Disease (VWD)

Three types exist, with Type 1 being the most common.
Type 2 has subtypes; Type 2A leads to low VWF affecting platelet function.
Type 3 is the most severe form, characterized by an almost complete absence of VWF.
Inherited through mutations; treatments include desmopressin for Type 1 and replacement therapy for severe cases.

Key Diagnostic Tests for VWD

CBC, VWF antigen test, ristocetin co-factor test, and specific factor assays are critical for diagnosis.
Prolonged bleeding and excessive bleeding during procedures are common symptoms.

Treatment and Contraindications

Desmopressin is effective for Type 1 and some Type 2 variants but contraindicated in Type 2B due to low platelet counts.
Aminocaproic acid and tranexamic acid are used in management; NSAIDs and blood thinners should be avoided in VWD patients.

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Description

This quiz focuses on the pharmacotherapy associated with coagulation disorders, covering their pathophysiology, inducing factors, and clinical presentations. Students will explore diagnostic methods and therapeutic outcomes in the context of hemostasis and coagulation management.