Erythrocyte and leucocyte enzymes in a case of paroxysmal nocturnal haemoglobinuria.
In a patient with paroxysmal nocturnal haemoglobinuria (PNH) enzymatic activities of erythrocytes and leucocytes were studied. Studies of autohaemolysis were also performed. The following erythrocytary enzymes were measured: Glucose-6-phosphate dehydrogenase (G-6-PD), pyruvate kinase (PK), glutathione reductase (GR), and acetylcholinesterase (AcChE). The following enzymes were measured in leucocytes: Adenosine deaminase, purine nucleoside phosphorylase, adenine phosphoribosyltransferase, hypoxanthine phosphoribosyltransferase and adenosine kinase. Normal activity of G-6-PD, GR and PK in erythrocytes was found. In leucocytes and lymphocytes activity of purine nucleoside phosphorylase was reduced. Auto-haemolysis in vitro was increased, which could not be compensated by addition of glucose or ATP.
D000110:Acetylcholinesterase / Q000097:blood; D000228:Adenine Phosphoribosyltransferase / Q000097:blood; D000243:Adenosine Deaminase / Q000097:blood; D000248:Adenosine Kinase / Q000097:blood; D000328:Adult; D004912:Erythrocytes / Q000201:enzymology*; D005954:Glucosephosphate Dehydrogenase / Q000097:blood; D005980:Glutathione Reductase / Q000097:blood; D006457:Hemoglobinuria, Paroxysmal / Q000201:enzymology*; D006801:Humans; D007041:Hypoxanthine Phosphoribosyltransferase / Q000097:blood; D007962:Leukocytes / Q000201:enzymology*; D008297:Male; D011683:Purine-Nucleoside Phosphorylase / Q000097:blood; D011770:Pyruvate Kinase / Q000097:blood
D005954:Glucosephosphate Dehydrogenase; D005980:Glutathione Reductase; D011683:Purine-Nucleoside Phosphorylase; D000228:Adenine Phosphoribosyltransferase; D007041:Hypoxanthine Phosphoribosyltransferase; D000248:Adenosine Kinase; D011770:Pyruvate Kinase; D000110:Acetylcholinesterase; D000243:Adenosine Deaminase