SJH Nursing Officer - 2019
Medical & Surgical Nursing
Easy

A hereditary hemolytic anaemia is?

Appeared in: SJH Nursing Officer - 2019

Explanation

  • Thalassemia is a group of inherited blood disorders passed from parents to children through genes (hereditary).
  • It is caused by mutations that reduce the synthesis of either the alpha or beta globin chains of hemoglobin.
  • This imbalance in globin chain production leads to defective red blood cell formation and also causes premature destruction (hemolysis) of red blood cells, resulting in anemia.
  • The search results confirm that intrinsic hemolytic anemias are often inherited and include thalassemia.

Why Other Options Were Wrong

  • Option A: Aplastic anemia is primarily a disorder of the bone marrow, where it fails to produce sufficient numbers of red cells, white cells, and platelets. It is not a hemolytic anemia (i.e., not caused by RBC destruction) and is usually acquired rather than hereditary.
  • Option C: Megaloblastic anemia is a nutritional deficiency anemia resulting from a lack of vitamin B12 or folate. This deficiency impairs DNA synthesis and leads to the formation of large, abnormal red blood cells (megaloblasts). It is not a hereditary or primarily hemolytic condition.
  • Option D: This option is incorrect because only Thalassemia among the choices is a hereditary hemolytic anemia. Aplastic anemia and Megaloblastic anemia do not fit this classification.

Related Visual

Visual explanation — Related Visual
Clinical Relevance
  • Nursing practice connection: This is primarily an exam-oriented knowledge point with limited direct bedside application, so retain Classification of Anemias as background academic context rather than a clinical decision trigger.
  • Nurses play a vital role in managing patients with thalassemia, which includes administering blood transfusions, managing iron chelation therapy to prevent iron overload (a common complication), and monitoring for disease complications.
  • Patient and family education is crucial, focusing on the genetic nature of the disease, the importance of adherence to treatment, and the availability of genetic counseling for family planning.
  • What if? If a patient with thalassemia develops a fever, it should be treated as a medical emergency. Splenic dysfunction or removal (common in thalassemia) increases the risk of life-threatening infections from encapsulated bacteria.
How to Approach the Question
  • First, break down the question's key terms: 'Hereditary' means it is passed down through genes from parents, and 'hemolytic' means it involves the breakdown or destruction of red blood cells.
  • Next, evaluate each option against these two criteria.
  • Analyze Option A (Aplastic anaemia): Is it hereditary? Usually not. Is it hemolytic? No, it's a production problem.
  • Analyze Option B (Thalassemia): Is it hereditary? Yes. Is it hemolytic? Yes. This option fits both criteria.
  • Analyze Option C (Megaloblastic anaemia): Is it hereditary? No, it's a nutritional deficiency. Is it hemolytic? Not primarily. This option is incorrect.
  • Since only one option correctly fits the description, 'All of the above' is also incorrect. Conclude that Thalassemia is the correct answer.
Concept Tested & Keywords
  • Concept Tested: Classification of Anemias
  • Stem keywords: hereditary, hemolytic anaemia
  • Lead-in keywords: is

Question ID

Q0rv5Vsu8w1ZEG58lsO9V6

Reference Book

E6 Physiology Essentials Sembulingam 10e Part 1 p. 104-106

E6 Pathology- ROBBINS & COTRAN PATHOLOGIC BASIS OF DISEASE 10TH Ed p. 646-648

E6 Parks TextBook of Preventive & Social Medicine part 2 — Subpart B (pp 233-449 of 464) p. 141-143

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