Erythropoiesis versus inflammation in Hereditary Spherocytosis clinical outcome

Objectives: This study aimed to evaluate the relationship between erythropoiesis and inflammation, in Hereditary Spherocytosis (HS) clinical outcome. Design and methods: We studied 26 controls and 82 HS patients presenting mild (n= 49) and severer (n= 33) HS forms. We evaluated plasma levels of EPO, sTfR, ferritin, iron, folic acid, vitamin B12, TNF-α, IFN-β, elastase and lactoferrin; leukocyte and reticulocyte counts and RPI were determined. Results: All HS patients showed significantly higher EPO, sTfR, reticulocytes and RPI but only mild HS presented normal hemoglobin levels; the positive significant correlations between EPO and sTfR, reticulocytes and RPI observed in mild HS were not observed in severer HS patients. HS patients presented with higher levels of neutrophils, TNF-α, IFN-β, elastase, lactoferrin and ferritin. Conclusions: Our data show HS as a disease linked to enhanced erythropoiesis that is disturbed in the more severe forms, to which inflammation may contribute, at least in part.