Hereditary spherocytosis is characterised by –
So, the core concept here is likely the pathophysiology of hereditary spherocytosis. The key points would be the defective RBC membrane proteins, like ankyrin, band 3, or spectrin. These proteins are part of the cytoskeleton, and their defects cause the cells to lose their shape and become spherocytes. This leads to increased osmotic fragility and splenic sequestration.
The correct answer options would probably include features like spherocytes on peripheral smear, anemia, jaundice, and splenomegaly. Also, the osmotic fragility test is a classic diagnostic tool. Maybe the options given include these. The user didn't list the options, but based on standard questions, possible distractors could be features of other hemolytic anemias like G6PD deficiency or thalassemia.
For the wrong options, they might include things like target cells (seen in thalassemia), elliptocytes (in hereditary elliptocytosis), or features of acquired conditions. Each of these would be incorrect because they relate to different disorders. For example, G6PD deficiency is an enzymatic defect, not a membrane defect.
The clinical pearl here is that hereditary spherocytosis is the most common inherited hemolytic anemia in Caucasians, and splenectomy is a treatment option. Also, the osmotic fragility test being positive is a key diagnostic point.
Putting it all together, the correct answer should mention the membrane defect, spherocytes, and the associated symptoms. The explanation should clarify why other options are incorrect by contrasting them with the pathophysiology of hereditary spherocytosis.
**Core Concept**
Hereditary spherocytosis (HS) is an **autosomal dominant hemolytic anemia** caused by **defects in red blood cell (RBC) membrane proteins** (e.g., ankyrin, spectrin, band 3). These defects disrupt the RBC cytoskeleton, leading to **spherocyte formation**, **increased osmotic fragility**, and **splenic sequestration** of abnormal cells.
**Why the Correct Answer is Right**
The correct answer must include **spherocytes on peripheral smear**, **hereditary inheritance pattern**, and **positive osmotic fragility test**. The membrane protein defects reduce RBC deformability, causing them to become rigid, spherical cells that are prematurely destroyed in the spleen. This results in **chronic hemolytic anemia**, **jaundice**, and **splenomegaly**.
**Why Each Wrong Option is Incorrect**
**Option A:** If it mentions **target cells**, this is incorrect. Target cells are seen in **liver disease** or **thalassemia**, not HS.
**Option B:** If it states **elliptocytes**, this is incorrect. Elliptocytes are characteristic of