Evaluation of the main regulators of systemic iron homeostasis in pyruvate kinase deficiency.

ABSTRACT: Iron homeostasis and dyserythropoiesis are poorly investigated in pyruvate kinase deficiency (PKD), the most common glycolytic defect of erythrocytes. Herein, we studied the main regulators of iron balance and erythropoiesis, as soluble transferrin receptor (sTfR), hepcidin, erythroferrone (ERFE), and erythropoietin (EPO), in a cohort of 41 PKD patients, compared with 42 affected by congenital dyserythropoietic anemia type II (CDAII) and 50 with hereditary spherocytosis (HS). PKD patients showed intermediate values of hepcidin and ERFE between CDAII and HS, and clear negative correlations between log-transformed hepcidin and log-EPO (Person's r correlation coefficient =  - 0.34), log-hepcidin and log-ERFE (r =  - 0.47), and log-hepcidin and sTfR (r =  - 0.44). sTfR was significantly higher

SUBMITTER: Zaninoni A 

PROVIDER: S-EPMC10020532 | biostudies-literature | 2023 Mar

REPOSITORIES: biostudies-literature