PMID: 7336157Jul 1, 1981Paper

Haem biosynthesis in refractory sideroblastic anaemia associated with the preleukaemic syndrome

Scandinavian Journal of Haematology
A V PasanenR Tenhunen

Abstract

The activities of 5 enzymes of the haem biosynthetic pathway and the protoporphyrin concentrations have been measured in peripheral red blood cells of 23 patients having a preleukaemic syndrome with refractory sideroblastic anaemia. A decreased delta-aminolaevulinic acid synthase (ALA-S) activity, an increased uroporphyrinogen I synthase activity and an increased red cell protoporphyrin concentration were consistent findings. Patients with abnormal leucocyte and/or platelet counts in the peripheral blood as well as patients with an excess of blast cells in the bone marrow had the lowest ALA-S activities. A further decrease in ALA-S activity was observed in 3 patients after leukaemic change in the disease. Patients having cytogenetic abnormalities showed no unique enzyme abnormalities. These results indicate that enzymatic disturbances of haem synthesis cannot be used as prognostic indicator of leukaemic transformation in refractory sideroblastic anaemia, but a very low ALA-S activity appears to accompany the development of a leukaemia in such patients.

References

Jan 1, 1979·Recent Results in Cancer Research. Fortschritte Der Krebsforschung. Progrès Dans Les Recherches Sur Le Cancer·M Doss
May 1, 1979·British Journal of Haematology·L Konopka, A V Hoffbrand
May 1, 1974·The Journal of Clinical Investigation·Y AokiF Takaku
Oct 1, 1972·The Journal of Clinical Investigation·L J StrandH S Marver
Mar 1, 1971·British Journal of Haematology·J M WhiteM A Ali
Feb 23, 1971·Biochimica Et Biophysica Acta·G Romeo, E Y Levin
Jan 1, 1980·The International Journal of Biochemistry·A V PasanenR Tenhunen

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Citations

Jan 1, 1989·European Journal of Haematology·L Volin
Jan 1, 1986·Scandinavian Journal of Haematology. Supplementum·R Lintula
Aug 8, 2006·Oncogene·M FontenayE Solary
Jan 1, 1986·Scandinavian Journal of Haematology. Supplementum·A Pasanen, R Tenhunen
Jan 1, 1986·Scandinavian Journal of Haematology. Supplementum·R Hast
Nov 26, 2002·Hematology·Peter L GreenbergNorbert Gattermann
Nov 1, 1992·European Journal of Haematology·T T Timonen, H Kauma
Apr 1, 1995·Journal of Cellular Physiology·K Muta, S B Krantz
Apr 1, 1987·British Journal of Haematology·T RuutuR Tenhunen
Oct 1, 1988·Biochemical Medicine and Metabolic Biology·N SchoenfeldA Atsmon
Oct 1, 1990·Biochemical Medicine and Metabolic Biology·R MametN Schoenfeld
Jan 1, 1988·Leukemia Research·L VolinR Tenhunen

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