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Pediatrics Lysosomal Storage Diseases 5b48523f

Bone marrow examination of a 2 year old child with massive splenomegaly and pancytopenia showed cells with wrinkled paper appearance of cytoplasm. What could be the possible diagnosis?

A
Niemann Pick disease
B
Gaucher disease
C
Acute Leukemia
D
Hereditary spherocytosis
High-Yield Explanation
Given scenario suggests Gaucher disease Gaucher disease is the most common lysosomal storage disorder with a defect of lysosomal enzyme acid b glucosidase. Gaucher cells are lipid laden macrophages that show a characteristic histologic appearance of "wrinkled tissue paper" of cytoplasm.

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