Successful Desensitization in a Patient with Hypersensitivity Reaction to Laronidase

Abstract

Mucopolysaccharidosis I (MPS I) is a rare, recessively inherited, lysosomal storage disorder caused by deficiency of the enzyme α-L-iduronidase. Scheie syndrome is the least severe form of MPS I. Patients can be diagnosed in the adolescent age group. Enzyme replacement therapy with laronidase is the best treatment option in Scheie syndrome and is generally well tolerated by patients. Anaphylaxis and other immediate hypersensitvity reactions to laronidase are very rare. Immediate hypersensitivity reaction to laronidase and successful desensitization with laronidase have only been described in 2 pediatric patients. We herein report the first case of a severe immediate hypersensitivity reaction to laronidase in an adult patient with MPS-I disease, who was successfully treated with a laronidase desensitization protocol.