5th February 2024

A 19-year-old female presents with abdominal distension, fatigue and epistaxis.

A 19-year-old female presents with abdominal distension, fatigue and epistaxis. Examination reveals a painless hepatosplenomegaly. Blood tests show an associated anaemia, leukopenia and thrombocytopenia. She reports a previous history of avascular necrosis of her right shoulder and left hip approximately three years ago, initially felt to be secondary to sports trauma. Mutations in the GBA gene were noted. 

Which of the following enzymes are likely defective?