Deficiency of coagulation factor VIII leads to bleeding disorder?

JIPMER Nursing Officer-2024

Pathology & Genetics

Easy

Appeared in: JIPMER Nursing Officer-2024

Explanation

Hemophilia A, also known as classic hemophilia, is the bleeding disorder caused by a deficiency or reduced activity of coagulation Factor VIII.
This condition is an X-linked recessive genetic disorder, which is why it primarily manifests in males.
The deficiency of Factor VIII impairs the intrinsic coagulation pathway, leading to characteristic symptoms like prolonged bleeding after trauma and spontaneous bleeding into joints (hemarthrosis) and muscles.
Laboratory tests for Hemophilia A typically show a prolonged activated partial thromboplastin time (APTT) with a normal prothrombin time (PT) and bleeding time.

Option B: Hemophilia B, also known as Christmas disease, is incorrect because it is caused by a deficiency of coagulation Factor IX, not Factor VIII.
Option C: Von Willebrand disease is caused by a deficiency or dysfunction of von Willebrand factor (vWF). Although vWF helps stabilize Factor VIII in the blood, and its deficiency can lead to reduced Factor VIII levels, the primary defect is not in Factor VIII itself.
Option D: Scurvy is a condition caused by a severe deficiency of Vitamin C (ascorbic acid). It leads to bleeding due to impaired collagen synthesis, which results in fragile blood vessels, but it is not a disorder of coagulation factors.

Visual 1: Diagram: The coagulation cascade, highlighting the specific role of Factor VIII in activating Factor X within the intrinsic pathway.
Visual 2: Table: A comparative chart of Hemophilia A, Hemophilia B, and Von Willebrand disease, detailing the deficient factor, inheritance pattern, typical symptoms, and key laboratory findings (APTT, PT, Bleeding Time, Factor Assays).

Clinical Relevance

Nursing practice connection: This is primarily an exam-oriented knowledge point with limited direct bedside application, so retain Coagulation Disorders and Factor Deficiencies as background academic context rather than a clinical decision trigger.
Nurses play a critical role in managing patients with Hemophilia A. This includes administering Factor VIII replacement therapy, managing acute bleeding episodes (RICE - Rest, Ice, Compression, Elevation), and providing pain management.
Patient and family education is a key nursing responsibility, focusing on recognizing early signs of a bleed, preventing injuries, and understanding the importance of prophylactic treatment to prevent joint damage.
A crucial patient safety measure is the avoidance of intramuscular (IM) injections and aspirin-containing medications, as these can provoke severe bleeding.

How to Approach the Question

This is a factual recall question that tests your knowledge of hematological disorders.
First, identify the key terms in the question stem: 'Deficiency' and 'coagulation factor VIII'.
The question asks you to link a specific factor deficiency to its corresponding disease.
Systematically review the options provided.
Recall that Hemophilia A is defined by Factor VIII deficiency.
Differentiate this from Hemophilia B (Factor IX deficiency), Von Willebrand disease (vWF deficiency), and Scurvy (Vitamin C deficiency) to confirm the correct answer.

Concept Tested & Keywords

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