LAMZEDE

What it is

LAMZEDE contains velmanase alfa . Each vial contains 10 mg of velmanase alfa, which after reconstitution yields a concentration of 2 mg per mL . The product is supplied as a powder for injection . Velmanase alfa is a recombinant form of human alpha-mannosidase with an amino acid sequence identical to the naturally occurring human enzyme . It is intended to supplement or replace natural alpha-mannosidase, an enzyme that catalyses the sequential degradation of hybrid and complex high-mannose oligosaccharides in the lysosome, reducing the amount of accumulated mannose-rich oligosaccharides .

Approved indications

— Enzyme replacement therapy for the treatment of non-central nervous system manifestations in patients with alpha-mannosidosis .

Dosing overview

The recommended dose regimen is 1 mg/kg of body weight administered once every week by intravenous infusion at a controlled speed . The total volume of infusion should be administrated over a minimum of 50 minutes for patients weighing up to 42 kg, and patients weighing 42 kg and greater should be infused at a maximum infusion rate of 25 mL/hour to control the protein load . A slower rate of infusion is recommended in patients new to treatment and in patients with previous infusion-related reactions .

Key safety warnings

Hypersensitivity reactions have been reported in patients in clinical studies and anaphylaxis is considered as a potential risk with LAMZEDE . Administration of velmanase alfa may result in an infusion-related reaction, including anaphylactic-type reactions, with symptoms including hypersensitivity, cyanosis, nausea, vomiting, pyrexia, chills, feeling hot, malaise, urticaria, anaphylactoid reaction and hyperhidrosis . Infusion-related reactions were reported in 13% of patients in clinical studies, all of mild or moderate severity, with 2 reported as a serious adverse reaction . Velmanase alfa does not cross the blood-brain-barrier and does not affect irreversible complications including skeletal deformities, disostosis multiplex, central nervous system manifestations and impaired cognitive function . Based on findings in animal reproduction studies, LAMZEDE may cause fetal harm when administered to a pregnant woman and is not recommended during pregnancy and in women of childbearing potential not using effective contraception, unless the potential benefits to the mother outweigh the potential risks, including those to the fetus .

Contraindications

Severe allergic reaction to the active substance or to any of the excipients .

PBS listing

LAMZEDE was recommended for PBS listing in December 2025 for the treatment of non-neurological manifestations of alpha-mannosidosis . The product is supplied as a 10 mg powder for injection vial . The PBAC recommended a lower price to achieve cost-effectiveness .

Regulatory history

LAMZEDE was first listed on the ARTG on 26 March 2025 . An AusPAR was published on 13 March 2025 for enzyme replacement therapy for the treatment of non-central nervous system manifestations in patients with alpha-mannosidosis . The PBAC deferred the application in July 2025 to seek further information on benefits, cost-effectiveness, and eligibility criteria . The product was recommended for listing by the PBAC in December 2025 for the treatment of non-neurological manifestations of alpha-mannosidosis . LAMZEDE is subject to additional monitoring in Australia to allow quick identification of new safety information .