Cooley's Anaemia is called?

CGHS SSC - 2018

Pathology & Genetics

Easy

Appeared in: CGHS SSC - 2018

Explanation

Cooley's Anaemia is the eponym for β-thalassemia major, the most severe form of beta-thalassemia.
Thalassemias are a group of inherited blood disorders characterized by a quantitative defect in hemoglobin synthesis, specifically the reduced production of one or more globin chains.
In β-thalassemia major, the synthesis of beta-globin chains is severely reduced or absent, leading to ineffective red blood cell production, severe anemia, and dependence on blood transfusions.
The condition typically manifests in early childhood with symptoms like pallor, failure to thrive, and hepatosplenomegaly.

Option B: Leukaemia is a type of cancer affecting the blood and bone marrow, characterized by the rapid production of abnormal white blood cells. It is a malignancy, not an inherited disorder of hemoglobin synthesis.
Option C: Haemophilia is a genetic bleeding disorder caused by a deficiency in specific clotting factors (most commonly Factor VIII or IX). It affects the body's ability to form blood clots, leading to prolonged bleeding, but it does not involve defects in hemoglobin production.
Option D: Purpura is not a disease itself but a symptom. It refers to purple-colored spots and patches on the skin, mucous membranes, and internal organs caused by bleeding from small blood vessels. It can result from various conditions, including platelet disorders (like ITP) or vasculitis, but it is not synonymous with Cooley's Anaemia.

Clinical Relevance

Nursing practice connection: This is primarily an exam-oriented knowledge point with limited direct bedside application, so retain Nomenclature of Hematological Disorders as background academic context rather than a clinical decision trigger.
Nurses play a vital role in managing patients with Cooley's Anaemia, which includes administering blood transfusions, managing iron chelation therapy, and monitoring for complications like iron overload, which can damage the heart, liver, and endocrine glands.
Patient and family education is a key nursing responsibility. This includes teaching about the genetic nature of the disease (autosomal recessive), the importance of adherence to treatment, and recognizing signs of complications.
What if? If a child with known Cooley's Anaemia presents with a fever, a nurse must act quickly. These patients, especially if they have had a splenectomy, are at high risk for severe infections. Immediate workup for sepsis is critical.

How to Approach the Question

First, identify the key term in the question stem, which is 'Cooley's Anaemia'.
Recognize that this is a factual recall question asking for the common medical term or classification for this eponym.
Systematically evaluate each option based on your knowledge of hematology.
Recall or deduce that 'Cooley's Anaemia' is a specific, severe form of an inherited anemia.
Eliminate Leukaemia (a cancer), Haemophilia (a clotting disorder), and Purpura (a symptom of bleeding).
This leaves Thalassaemia, which is the correct category of inherited hemoglobin disorders to which Cooley's Anaemia (β-thalassemia major) belongs.

Concept Tested & Keywords

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