Phosphorylase activity in chronic erythremic myelosis.
Phosphorylase activity was detected in the cytoplasm of erythroid precursors of 6 of 7 patients with chronic erythremic myelosis (Di Guglielmo syndrome), in proerythroblasts and megaloblasts from 3 patients with pernicious anemia and in 2 patients with severe folate deficiency in neoplastic lymphocytes from 2 patients with acute lymphoblastic leukemia, and in 1 patient with leukemic lymphosarcoma. In all of these patients, most of the erythroid precursors and/or neoplastic lymphocytes contained increased amounts of glycogen when stained with the PAS reagent. Phosphorylase activity was not detected in erythroid precursors obtained from 6 presumed normal individuals or from 3 of 7 patients with a variety of other types of anemia in which the erythroid precursors were PAS-negative. Similarly, phosphorylase activity was absent in lymphocytes obtained from presumed normal individuals. Although the mechanisms responsible for the pathogenesis of PAS positivity are unclear, it is possible that the increased phosphorylase activity found in cells that are PAS-positive may reflect a disorder in the biosynthetic pathway of glycogen.
D000743:Anemia, Hemolytic / Q000201:enzymology; D000744:Anemia, Hemolytic, Autoimmune / Q000201:enzymology; D000752:Anemia, Pernicious / Q000201:enzymology; D001853:Bone Marrow / Q000201:enzymology* / Q000473:pathology; D001854:Bone Marrow Cells; D004900:Erythroblasts / Q000201:enzymology; D005494:Folic Acid Deficiency / Q000201:enzymology; D006003:Glycogen / Q000302:isolation & purification; D006801:Humans; D007938:Leukemia / Q000201:enzymology; D004915:Leukemia, Erythroblastic, Acute / Q000201:enzymology*; D007945:Leukemia, Lymphoid / Q000201:enzymology; D006005:Phosphorylases / Q000302:isolation & purification*; D013103:Spherocytosis, Hereditary / Q000201:enzymology; D013194:Staining and Labeling
D006003:Glycogen; D006005:Phosphorylases