Phosphorylase activity in chronic erythremic myelosis

Area of Science:

• Hematology
• Biochemistry
• Cell Biology

Background:

• Glycogen accumulation in blood cells can be indicative of metabolic disorders.
• Phosphorylase is a key enzyme in glycogenolysis.

Purpose of the Study:

• To investigate the presence and significance of phosphorylase activity and glycogen accumulation in various hematological conditions.

Main Methods:

• Cytoplasmic phosphorylase activity assays.
• Periodic acid-Schiff (PAS) staining for glycogen detection.
• Analysis of erythroid precursors and neoplastic lymphocytes.

Main Results:

• Phosphorylase activity was detected in erythroid precursors of patients with chronic erythremic myelosis (Di Guglielmo syndrome), pernicious anemia, and severe folate deficiency.
• Neoplastic lymphocytes from patients with acute lymphoblastic leukemia and leukemic lymphosarcoma also showed phosphorylase activity.
• Increased glycogen content (PAS-positive staining) was observed in these cells.
• Phosphorylase activity was absent in normal individuals' blood cells and in some anemia patients with PAS-negative cells.

Conclusions:

• The presence of phosphorylase activity and increased glycogen in specific patient cell types suggests a potential disorder in glycogen biosynthesis.
• These findings may contribute to understanding the pathogenesis of PAS positivity in hematological disorders.