A bleeding disorder characterized by deficiency of factor VIII and IX termed as?

UPPSC 2023

Medical & Surgical Nursing

Easy

Appeared in: UPPSC 2023

Explanation

Hemophilia is the correct medical term for a group of inherited bleeding disorders caused by a deficiency in specific clotting factors.
The question specifically mentions Factor VIII and Factor IX, which correspond to the two most common types of the disorder: Hemophilia A (Factor VIII deficiency) and Hemophilia B (Factor IX deficiency).
This lack of clotting factors impairs the coagulation cascade, which is the body's process for forming a stable blood clot, leading to prolonged bleeding after injury or spontaneous bleeding.

Option A: Purpura refers to the purple-colored spots that appear on the skin due to subcutaneous bleeding. It is a clinical sign or symptom, not a specific disease defined by a deficiency of clotting factors VIII or IX.
Option C: Thrombocytopenia is a condition characterized by a low platelet count. Platelets are responsible for forming the initial plug to stop bleeding, which is a different mechanism than the coagulation cascade involving protein factors like VIII and IX.
Option D: Leukemia is a type of cancer affecting blood-forming cells in the bone marrow. While it can cause bleeding complications by suppressing the production of platelets, it is a malignancy and not a primary inherited clotting factor deficiency.

Clinical Relevance

Nursing practice connection: This is primarily an exam-oriented knowledge point with limited direct bedside application, so retain Hereditary Bleeding Disorders and Coagulation Factors as background academic context rather than a clinical decision trigger.
Accurate diagnosis is critical for treatment. Nurses must know that Hemophilia A is treated with Factor VIII concentrate, while Hemophilia B requires Factor IX concentrate. Administering the wrong factor is ineffective.
Nursing care for patients with hemophilia focuses on preventing injury, managing bleeding episodes (e.g., RICE for joint bleeds), pain management, and patient/family education on recognizing signs of serious internal bleeding (e.g., headache, abdominal pain).
What if? If the patient had a prolonged bleeding time but a normal platelet count and normal Factor VIII/IX levels, the nurse might suspect von Willebrand Disease. This is the most common inherited bleeding disorder and involves a defect in von Willebrand Factor (vWF), which helps platelets stick together and also stabilizes Factor VIII.

How to Approach the Question

Identify the key concepts in the question stem: 'bleeding disorder' and 'deficiency of factor VIII and IX'.
This is a factual recall question requiring knowledge of specific hematologic diseases.
Systematically evaluate each option against the definition provided in the stem.
Recall that 'Purpura' is a symptom (skin discoloration from bleeding).
Recall that 'Thrombocytopenia' is a low platelet count, not a factor deficiency.
Recall that 'Leukemia' is a cancer.

Concept Tested & Keywords

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E6 PATHOLOGY QUICK REVIEWBased on Harsh Mohan Textbook of PATHOLOGY p. 133-135

E6 Pathology- ROBBINS & COTRAN PATHOLOGIC BASIS OF DISEASE 10TH Ed pp. 671-673, 673-675