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Question

Which of the following is characterised by an increase in osmotic fragility of red cells?

The correct answer is
Hereditary spherocytosis

Understanding Red Cell Osmotic Fragility

Osmotic fragility refers to the susceptibility of red blood cells (RBCs) to lysis (bursting) when placed in solutions of varying osmotic pressure, typically saline. An increase in osmotic fragility means that RBCs lyse more readily, even in relatively concentrated salt solutions.

Analyzing Conditions and Osmotic Fragility

Let's examine how each condition affects RBC osmotic fragility:

  • Thalassaemia major: This condition involves defective hemoglobin synthesis. RBCs are often microcytic (small) and hypochromic (pale). Osmotic fragility is typically normal or even decreased, as smaller cells are more resistant to lysis.
  • Iron deficiency anaemia: Similar to thalassemia, iron deficiency results in microcytic, hypochromic RBCs. These cells generally exhibit decreased osmotic fragility.
  • Hereditary spherocytosis: This is an inherited disorder affecting the RBC membrane structure. Genetic defects lead to the formation of spherocytes – spherical RBCs lacking the normal biconcave disc shape. Spherocytes are less flexible and have a higher surface area-to-volume ratio, making them significantly more vulnerable to lysis in osmotic gradients. Consequently, hereditary spherocytosis is characterized by a marked increase in osmotic fragility.
  • Pyridoxine deficiency: Deficiency in Vitamin B6 can lead to certain types of anemia (e.g., sideroblastic anemia). The effect on osmotic fragility is generally minimal, often considered normal.

Therefore, the condition characterized by an increase in osmotic fragility of red cells is Hereditary spherocytosis.

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Important Questions from Anemia

  1. A 39-year-old female presents with severe autoimmune hemolytic anemia. Which of the following should be the initial step in its management?
  2. Which one of the following is a cause of relative erythrocytosis?
  3. Which one of the following conditions characteristically may present with a triad of haemolysis, pancytopenia and venous thrombosis?
  4. Which one of the following is associated with low MCV of red blood cells?
  5. Which one of the following is the primary underlying mechanism of haemolysis in paroxysmal nocturnal haemoglobinuria (PNH)?
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