LYSOSOMAL STORAGE DISEASES Flashcards
flawed degradation of phagocytized material and buildup of undigested substrates within lysosomes
LYSOSOMAL STORAGE DISEASES
-The macrophages are particularly prone to accumulate undegraded lipid products, which subsequently leads to an expansion of the reticuloendothelial tissue
LIPID STORAGE DISEASES/ SPHINGOLIPIDOSES
-Are qualitative disorders involving monocytes and macrophages
LIPID STORAGE DISEASES/ SPHINGOLIPIDOSES
-most common of the lysosomal lipid storage diseases ; at least 1 in 17 Ashkenazi Jews are carriers
GAUCHER’S DISEASE
-It is an autosomal recessive disorder caused by a defect or deficiency in the catabolic enzyme beta-glucocerebrosidase
GAUCHER’S DISEASE
can be found in bone marrow of some patients with thalassemia, chronic myeloid leukemia, acute lymphoblastic leukemia, non-Hodgkin lymphoma, and plasma cell neoplasms
Pseudo-Gaucher cells