Which of the following is a hereditary hemolytic anemia?

RUHS, Jaipur, M.Sc Nursing Entrance Exam-2016

Pathology & Genetics

Easy

Appeared in: RUHS, Jaipur, M.Sc Nursing Entrance Exam-2016

Explanation

Thalassemia is correctly identified as a hereditary hemolytic anemia.
It is an inherited (autosomal recessive) genetic disorder that affects the synthesis of hemoglobin.
This defect results in the production of abnormal, fragile red blood cells (RBCs) that are prone to premature destruction (hemolysis), leading to anemia.

Option A: Aplastic anemia is a hypoproliferative anemia, not a hemolytic one. The primary problem is the bone marrow's failure to produce sufficient blood cells.
Option B: Pernicious anemia is a specific type of megaloblastic anemia caused by an autoimmune condition leading to Vitamin B12 deficiency. It is a problem of RBC maturation, not a primary hereditary hemolytic disorder.
Option C: Megaloblastic anemia is a broad category of anemia characterized by large, immature RBCs due to impaired DNA synthesis, typically from Vitamin B12 or folate deficiency. While the cells can be fragile, the primary defect is in maturation, not a specific inherited hemolytic process.

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 key role in managing patients with Thalassemia, which often involves administering regular blood transfusions.
A critical nursing intervention is monitoring for iron overload (hemosiderosis), a common complication of frequent transfusions. This involves assessing for signs of organ damage and administering iron-chelating agents like Deferoxamine as prescribed.
Patient education is vital, including genetic counseling for families, understanding the signs of anemia, and the importance of adhering to treatment.

How to Approach the Question

First, break down the question's keywords: 'hereditary' (meaning genetic, passed down in families) and 'hemolytic anemia' (meaning premature destruction of red blood cells).
Your goal is to find the option that is both a genetic condition and involves the destruction of red blood cells.
Evaluate Option A (Aplastic anemia): Recall this is about the bone marrow not producing cells. This is a production problem, not a destruction (hemolytic) problem. Eliminate.
Evaluate Option B and C (Pernicious/Megaloblastic anemia): Recall these are related to Vitamin B12/folate deficiency, which affects RBC maturation and size. This is a maturation problem, not a primary hereditary hemolytic issue. Eliminate.
Evaluate Option D (Thalassemia): Recall this is a well-known genetic disorder affecting hemoglobin. This defect makes the RBCs fragile and leads to their destruction. This matches both 'hereditary' and 'hemolytic'.
Confirm that Thalassemia is the best fit, as it is a classic example of a hereditary hemolytic anemia.

Concept Tested & Keywords

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