Fig. 1.

Immunomodulation therapy (IMT) protocol and anti-alglucosidase alfa antibody titers. Rituximab (R) 375 mg/m2/dose IV; after Wk 25, the patient received one additional administration in Wk 37. Methotrexate (M) 0.5 mg/kg weekly (orally or G-tube) to maintain absolute neutrophil count <500/μL and platelet count <50,000/μL. Discontinued in Wk 46. Intravenous immunoglobulin (IVIG) 500 mg/kg IV every 4 weeks. Monthly administrations continued till Wk 46. Alglucosidase alfa (ERT) restarted at 5 days of IMT at 20 mg/kg every other week (EOW). Dose increased to 40 mg/kg EOW in Wk 10 of IMT (continuing). After Wk 25, anti-alglucosidase alfa (ERT) antibody titers remained undetectable (<1:100)