TY - CHAP M1 - Book, Section TI - Gaucher Disease and Related Lysosomal Storage Diseases A1 - Lichtman, Marshall A. A1 - Kaushansky, Kenneth A1 - Prchal, Josef T. A1 - Levi, Marcel M. A1 - Burns, Linda J. A1 - Linch, David C. PY - 2022 T2 - Williams Manual of Hematology, 10e AB - These are hereditary disorders in which one or more tissues become engorged with specific lipids because of deficiencies of the lysosomal enzymes required for hydrolysis of one of the glycosidic bonds. The type of lipid and its tissue distribution have a characteristic pattern in each disorder.In Gaucher disease (the most common disorder) and Niemann-Pick disease, major clinical manifestations result from macrophage accumulation of glucocerebroside and sphingomyelin, respectively, leading to their massive expansion in tissues. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - hemonc.mhmedical.com/content.aspx?aid=1189334143 ER -