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Pathology General 65defc0f

True statements regarding hereditary spherocytosis are all of the following except: March 2012

A
Splenomegaly
B
Anemia
C
Spherocytosis is a pathognomic finding
D
It is due to intrinsic defect in RBC membrane skeleton
High-Yield Explanation
Ans: C i.e. Spherocytosis is a pathognomic finding Hereditary spherocytosis The characteristic clinical features are anemia, moderate splenomegaly (500-1000 gm), and jaundice. The most specific morphologic finding of hereditary spherocytosis is spherocytosis but spherocytosis is not pathognomic, since other forms of membrane loss, such as in autoimmune haemolytic anemias, also cause the formation of spherocytes Hereditary spherocytosis is an inherited disorder caused by intrinsic defects in the RBC membrane skeleton that render RBC spheroid, less deformable, and vulnerable to splenic sequestration and destruction.

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