title: Enzymes of purine metabolism in hairy cell leukemia.
abstract: Differences in activities of the purine degradative enzymes, adenosine deaminase (ADA), purine nucleoside phosphorylase (PNP), and 5'-nucleotidase (5'NT), have been observed among different classes of lymphoid malignancies. Recent studies have shown that hairy cell leukemia (HCL) may respond to treatment with the ADA inhibitor, 2-deoxycoformycin. This study demonstrates that the cells of HCL have significantly lower levels of ADA and 5'NT (P always less than 0.01) when compared to levels in normal B- or T-lymphocytes, but have higher levels of PNP (P less than 0.001 for both comparisons). Recent studies have shown that when treated with 12-O-tetradecanoyl-phorbol-13-acetate (TPA), cells of B-cell chronic lymphatic leukemia (B-CLL) acquire phenotypic characters of HCL. The authors have therefore also investigated the changes in enzyme pattern of B-CLL after incubation with TPA B-CLL cells are characterized by low levels of ADA, PNP, and 5'-NT, but TPA caused a marked increase in PNP activity (P less than 0.001, t test for paired samples), a pattern similar to HCL. The results from biochemical studies are thus in accordance with the hypothesis that HCL cells are more mature than B-CLL cells. The special enzyme profile of HCL suggests that a PNP inhibitor might also be effective in the treatment of this disease.
mesh_terms: D015720:5'-Nucleotidase; D000619:Aminohydrolases / Q000032:analysis; D002478:Cells, Cultured; D005455:Fluorescent Antibody Technique; D006801:Humans; D007943:Leukemia, Hairy Cell / Q000201:enzymology*; D007945:Leukemia, Lymphoid / Q000201:enzymology*; D008213:Lymphocyte Activation; D008214:Lymphocytes / Q000187:drug effects / Q000201:enzymology*; D009708:Nucleotidases / Q000032:analysis; D010641:Phenotype; D011683:Purine-Nucleoside Phosphorylase / Q000032:analysis; D011687:Purines / Q000378:metabolism*; D013755:Tetradecanoylphorbol Acetate / Q000494:pharmacology
chemical_list: D011687:Purines; D011683:Purine-Nucleoside Phosphorylase; D009708:Nucleotidases; D015720:5'-Nucleotidase; D000619:Aminohydrolases; C018774:adenine deaminase; D013755:Tetradecanoylphorbol Acetate