Deficiency of clotting factor IX leads to?

Appeared in: NHM UP Staff Nurse - 2016

Explanation

A deficiency in clotting factor IX causes a specific bleeding disorder known as Hemophilia B.
Hemophilia B is also commonly referred to as Christmas disease.
The name 'Christmas disease' comes from the first patient diagnosed with this specific factor deficiency, Stephen Christmas, in 1952.
Clinically and in terms of inheritance (X-linked recessive), it is very similar to Hemophilia A, but it requires different treatment (infusions of Factor IX).

Option A: This is a general term. While Factor IX deficiency is a type of hemophilia (Hemophilia B), 'Christmas disease' is the more specific name for this condition.
Option B: Thrombocytopenia is a condition characterized by a low number of platelets (thrombocytes) in the blood. It is a disorder of primary hemostasis, not a deficiency of a specific clotting factor in the coagulation cascade.
Option D: Thalassemia is a group of inherited blood disorders that affect the body's ability to produce hemoglobin, the protein in red blood cells that carries oxygen. It causes anemia and is unrelated to clotting factors.

Clinical Relevance

Nursing practice connection: This is primarily an exam-oriented knowledge point with limited direct bedside application, so retain Coagulation Disorders as background academic context rather than a clinical decision trigger.
Nurses must be able to differentiate between Hemophilia A and B because treatment is factor-specific. Administering the wrong factor concentrate is ineffective and wastes critical resources.
Key nursing interventions for a patient with Christmas disease include preventing injury, administering Factor IX concentrate as prescribed, avoiding intramuscular injections and medications that affect platelet function (like aspirin and NSAIDs), and providing education on managing bleeds.
What if? If the patient had a deficiency of Factor VIII instead of Factor IX, the diagnosis would be Hemophilia A (Classic Hemophilia), and the treatment would be infusions of Factor VIII concentrate.

How to Approach the Question

First, identify the key terms in the question: 'deficiency' and 'clotting factor IX'.
This is a factual recall question that requires knowledge of specific hematological disorders.
Recall or look up the diseases associated with deficiencies of different clotting factors.
Associate Factor IX deficiency with its specific names: Hemophilia B and Christmas disease.
Evaluate the options. 'Hemophilia' is a broad term. 'Thrombocytopenia' and 'Thalassemia' are different types of blood disorders.
Select the most specific and accurate name for Factor IX deficiency, which is 'Christmas disease'.

Concept Tested & Keywords

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E6 PATHOLOGY QUICK REVIEWBased on Harsh Mohan Textbook of PATHOLOGY pp. 133-135, 134-136

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

Practise the full NHM UP Staff Nurse - 2016

Attempt every question from this paper in a timed mock, then review the full solution for each one.