Purine degradative enzymes in the malignant cells of patients with B-cell leukemia

  • A. D. Ho
  • B. Dörken
  • W. Hunstein
  • A. V. Hoffbrand
Part of the Developments in Oncology book series (DION, volume 32)


The purine degradative enzymes adenosine deaminase (ADA), purine nucleoside phosphorylase (PNP) and ecto-5’-nucleotidase (5-NT) seem to play an important role in the development of both T- and B-lymphocytes [1–4]. Inherited deficiencies of ADA and PNP are associated with various immune defects. Furthermore remarkable differences in enzyme activities of these purine enzymes and terminal deoxynucleotidyl transferase (TdT) have been reported among immunologic subclasses of acute lymphocytic leukemia (ALL) [5–8]. Studies of these enzymes in normal T-cell precursors have shown that different thymic subsets in different stages of maturation are characterized by specific enzyme patterns with a fall in TdT and ADA activities and a rise in PNP and 5’NT activities during T-cell maturation [9, 10]. Further investigations have shown that the enzyme patterns of malignant T-cells are comparable to the enzymatic make up of normal T-lymphocytes in defined stages of development [11, 12]. For example, the blasts of T-ALL have high levels of TdT and ADA activities but low levels of PNP and 5’NT, an enzyme pattern which corresponds to that of prothymocytes, their normal counterparts.


Terminal Deoxynucleotidyl Transferase Purine Nucleoside Phosphorylase Enzyme Pattern Prolymphocytic Leukemia Enzyme Terminal Deoxynucleotidyl Transferase 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Giblett ER, Anderson JE, Cohen F et al. (1972). Adenosine-deaminase deficiency in two patients with severely impaired cellular immunity. Lancet 2: 1067–1069.PubMedCrossRefGoogle Scholar
  2. 2.
    Giblett ER, Ammann AJ, Wara DW et al. (1975). Nucleoside-phosphorylase deficiency in a child with severely defective T-cell immunity and normal B-cell immunity. Lancet 1: 10101013.Google Scholar
  3. 3.
    Johnson SM, North ME, Asherson GL et al. (1977). Lymphocyte purine 5’-nucleotidase deficiency in primary hypogammaglobulinaemia. Lancet 1: 168–170.PubMedCrossRefGoogle Scholar
  4. 4.
    Barton RW and Goldschneider I (1979). Nucleotide-metabolizing enzymes and lymphocyte differentiation. Mol Cell Biochem 28: 135–147.PubMedCrossRefGoogle Scholar
  5. 5.
    Coleman MS, Greenwood MF, Hutton JJ et al. (1978). Adenosine deaminase, terminal deoxynucleotidyl transferase ( TdT) and surface markers in childhood acute leukemia. Blood 52: 1125–1131.Google Scholar
  6. 6.
    Blatt J., Reaman GH, Levin N and Poplack DG (1980). Purine nucleoside phosphorylase activity in acute lymphoblastic leukemia. Blood 56: 380–382.PubMedGoogle Scholar
  7. 7.
    Ganeshaguru K, Lee N, Llewellin P et al. (1981). Adenosine deaminase concentrations in leukaemia and lymphoma: A relation to cell phenotypes. Leuk Res 5: 215–222.Google Scholar
  8. 8.
    Sylwestrowicz TA, Ma DDF, Murphy PP et al. (1982). 5’Nucleotidase, adenosine deaminase and purine nucleoside phosphorylase activities in acute leukaemia. Leuk Res 6: 475–482.PubMedCrossRefGoogle Scholar
  9. 9.
    Barton RW and Goldschneider I (1979). Nucleotide-metabolizing enzymes and lymphocyte differentiation. Mol Cell Biochem 28: 135.PubMedCrossRefGoogle Scholar
  10. 10.
    Ma DDF, Sylwestrowicz TA, Granger S et al. (1982). Distribution of terminal deoxynucleotidyl transferase and purine degradative and synthetic enzymes in subpopulations of human thymocytes. J Immunol 129: 1430–1435.PubMedGoogle Scholar
  11. 11.
    Ma DDF, Massaia M, Sylwestrowicz TA et al. (1983). Comparison of purine degradative enzymes and terminal deoxynucleotidyl transferase in T-cell leukaemias and in normal thymic and post-thymic T-cells. Brit J Haematol 54: 451–457.CrossRefGoogle Scholar
  12. 12.
    Van Laarhoven JPRM, Spierenburg GT, Bakkeren JAJM et al. (1983). Purine metabolism in childhood acute lymphoblastic leukemia: Biochemical markers for diagnosis and chemotherapy. Leuk Res 7: 407–420.Google Scholar
  13. 13.
    Massaia M, Ma DDF, Sylwestrowicz TA et al. (1982). Enzymes of purine metabolism in human peripheral lymphocyte subpopulations. Clin Exp Immunol 50: 148–154.PubMedGoogle Scholar
  14. 14.
    Rowe M, de Gast CG, Platts-Mills TAE et al. (1979). 5’Nucleotidase of B- and T-lymphocytes isolated from human peripheral blood. Clin Exp Immunol 36: 97–101.PubMedGoogle Scholar
  15. 15.
    Lennert K (Ed.) (1978). Malignant Lymphomas Other than Hodgkin’s Disease. Berlin, Heidelberg, New York: Springer-Verlag.Google Scholar
  16. 16.
    Ezdinly EZ and Nanus DM (1983). B-lymphoproliferative disorders: A proposed unified pathogenetic pathway. Hematol Oncol 1: 297–319.CrossRefGoogle Scholar
  17. 17.
    Blatt J, Reaman G and Poplack D (1980). Biochemical markers in lymphoid malignancies. N Engl J Med 303: 918–922.PubMedCrossRefGoogle Scholar
  18. 18.
    Blatt J, Bunn PA, Carney DD et al. (1982). Purine pathway enzymes in the circulating malignant cells of patients with cutaneous T-cell lymphoma. Brit J Haematol 52: 97–104.CrossRefGoogle Scholar
  19. 19.
    Godai T and Funderud S (1982). Human B-cell neoplasms in relation to normal B-cell differentiation and maturation processes. Adv Cancer Res 36: 211–255.CrossRefGoogle Scholar
  20. 20.
    Johnstone AP (1982). Chronic lymphocytic leukaemia and its relationship to normal Blymphopoiesis. Immunol Today 3: 343–348.CrossRefGoogle Scholar
  21. 21.
    Rowe M, de Gast CG, Platts-Mills TAE et al. (1980). Lymphocyte 5’-Nucleotidase in primary hypogammaglobulinaemia and cord blood. Clin Exp Immunol 39: 337–343.PubMedGoogle Scholar
  22. 22.
    Kramers MTC, Catovsky D, Cherchi M and Galton DAG (1977). 5’Nucleotidase in cord blood lymphocytes. Leuk Res. 1: 279–281.CrossRefGoogle Scholar

Copyright information

© Martinus Nijhoff Publishers, Boston 1985

Authors and Affiliations

  • A. D. Ho
    • 1
  • B. Dörken
    • 1
  • W. Hunstein
    • 1
  • A. V. Hoffbrand
    • 2
  1. 1.Medizinische Universitäts-PoliklinikHeidelbergGermany
  2. 2.Department of HaematologyRoyal Free Hospital and School of MedicineLondonUK

Personalised recommendations