Abstract
In patients with sickle cell syndromes, EP levels may be markedly elevated. In 249 patients with sickle cell syndromes, we measured EP, ALAD, and ALAD after reactivation with dithiothreitol (ALADSH), in parallel with reticulocytes and pyruvate kinase (PK), two age-dependent parameters. EP, ALAD, ALADSH, reticulocytes and PK were much higher than normal in all patients, with the most marked elevation in patients with homozygous sickle cell disease. In many cases, the elevation of EP resulted from free protoporphyrin base, rather then Zn-protoporphyrin. The logarithm of EP was correlated both with reticulocytes and with the logarithm of PK; the regression lines intersected the normal ranges for EP and reticulocytes and for EP and PK respectively. For ALAD and ALADSH, there were similar correlations; however, the regression lines remained well above the normal ranges without intersecting them. The in vivo t1/2 in the erythrocytes of both ALAD and ALADSH were estimated on discontinuous gradients of arabino-galactane at 43 days, indicating an age-dependent loss of enzyme activity, and not just oxidation of SH-groups with time. The in vivo t1/2 of EP is 60 days (JCI 56: 1519, 1975). These data indicate that both EP and ALAD are increased in sickle cell syndromes as a result of young red cell age. However, the higher ALAD values and the unusual protoporphyrin species suggest additional factors, probably some degree of dyserythropoiesis or the presence of large numbers of normoblasts in these asplenic individuals.
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Piomelli, S., Seaman, C., Carriero, D. et al. ELEVATION OF δ -AMINOLEVULINIC ACID DEHYDRATASE (ALAD) AND ERYTHROCYTE PORPHYRINS (EP) IN SICKLE CELL SYNDROMES. Pediatr Res 21 (Suppl 4), 304 (1987). https://doi.org/10.1203/00006450-198704010-00824
Issue Date:
DOI: https://doi.org/10.1203/00006450-198704010-00824