Abstract

Porphyrin metabolism was studied in 21 children of both sexes suffering from acute lymphoblastic leukaemia (ALL) and 34 adult patients of different ages and sexes suffering from ALL (n = 14), non-Hodgkin's lymphoma (NHL), n = 14, or Hodgkin's disease (HD), n = 6. In addition, two groups of healthy children (n = 14), and adults (n = 17) were studied for comparison. It was apparent from this study that the activity of uroporphyrinogen-1-synthetase (URO-1-S, E.C. 4.3.1.8) was highly significantly activated in the blood of children, while the activities of blood 5-aminolevulinic acid dehydrase (E.C. 4.2.1.24) and ferrochelatase (E.C. 4.99.1.1.), as expressed by protoporphyrin/haem ratio, were inhibited in those children. Also, free erythrocyte total porphyrins were increased, while the haem content was reduced. The concentrations of 5-aminolevulinic acid, coproporphyprin and uroporphyrin were increased in the urine of children with ALL. On the other hand, some dramatic changes were found in porphyrin metabolism in adult patients suffering from ALL, NHL and HD. The aforementioned disturbances were discussed in the light of some factors which may affect the enzymatic activities in the synthesis of porphyrins.

Highlights

  • Summ- Porphvnn metabolism was studied in 21 children of both sexes suffering from acute lymphoblastic leukaemia (ALL) and 34 adult patients of different ages and sexes suffering from ALL (n = 14). nonHodgkin's lyxmphoma (NHL). n = 14. or Hodgkin's disease (HD). n = 6

  • The present study addresses the question of whether porphyrin metabolism is perturbed in certain malignant diseases such as acute lymphoblastic leukaemia. non-Hodgkin's lymphoma and Hodgkin's disease

  • The present results indicate that the free erythrocyte porphyrins are significantly increased in children with ALL and in adults with ALL, NHL and HD: this means that porphyria may be a finding accompanying ALL

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Summary

Introduction

Summ- Porphvnn metabolism was studied in 21 children of both sexes suffering from acute lymphoblastic leukaemia (ALL) and 34 adult patients of different ages and sexes suffering from ALL (n = 14). The present study addresses the question of whether porphyrin metabolism is perturbed in certain malignant diseases such as acute lymphoblastic leukaemia. Malik and Lugaci (1987) observed that endogenous porphyrin biosynthesis in Friend erythroleukaemic cells was induced by supplementation of 5-aminolevulinic acid. 5-aminolevulinic acid dehydrase activity is markedly higher in reticulocytes than in erythrocytes This increase in activity has been demonstrated during erythroid differentiation of Mouse Friend Virus transformed erythroleukaemia cells This work was confirmed by the studies of Walters et al (1967) who found that immature cells from patients with acute leukaemia could utilise ALA and protoporphyrin for haem synthesis. This work was confirmed by the studies of Walters et al (1967) who found that immature cells from patients with acute leukaemia could utilise ALA and protoporphyrin for haem synthesis. whereas leukocytes from healthy adults lacked this capacity

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