Imiglucerase for Injection is indicated for long-term enzyme replacement therapy in patients diagnosed with non-neuropathic (Type I) and chronic neuropathic (Type II) Gaucher disease who have significant clinical manifestations of non-neurological lesions.

The clinical manifestations of non-neurological lesions in Gaucher disease include one or more of the following conditions:

1. Anemia excluding that caused by other reasons, such as iron deficiency anemia.

2. Thrombocytopenia.

3. Bone disease excluding that caused by other reasons, such as vitamin D deficiency.

4. Hepatomegaly or splenomegaly.

Dosing

Range: 2.5 units/kg thrice weekly to 60 units/kg every 2 weeks.

Titration: Adjust based on disease severity and therapeutic goals.

Administration Protocol

Reconstitution:

Add 10.2 mL Sterile Water to each 400-unit vial; roll/tilt to dissolve (avoid shaking).

Final concentration: 40 units/mL.

Dilution:

Dilute with 0.9% NaCl to 100–200 mL (100 mL for patients <18 kg).

Infusion:

≥18 kg: 1–2 hours.

<18 kg: 2 hours.

Filtration: Use in-line 0.2 μm filter if needed.

Premedication

For hypersensitivity-prone patients: Antihistamines/corticosteroids.