РЕДКОЕ НАБЛЮДЕНИЕ РЕАКЦИИ ГИПЕРЧУВСТВИТЕЛЬНОСТИ НА ПРЕПАРАТЫ ДЛЯ ФЕРМЕНТНОЙ ЗАМЕСТИТЕЛЬНОЙ ТЕРАПИИ У РЕБЕНКА С БОЛЕЗНЬЮ ГОШЕ 3-го ТИПА

Abstract

Gaucher disease (GD) is an autosomal recessive disease that leads to excessive accumulation of glucocerebroside in the cells of the reticuloendothelial system, which leads to multiple organ damage with the development of cytopenia, hepatosplenomegaly, bone and neurological pathology. An effective and safe enzyme replacement therapy has been developed for the treatment of GD. However, there are rare cases of hypersensitivity reactions presented in the literature, requiring individualization of patient management with the search for alternative ways to continue therapy and prevent side effects. We present a rare clinical case of intolerance to imiglucerase and velaglucerase alfa in a child with GD type 3 and possible methods for continuing treatment, taking into account the hypersensitivity reaction.