Lipid profile in beta-thalassemia intermedia patients: correlation with erythroid bone marrow activity.
Lipid abnormalities, including low levels of all fractions of serum lipids, have been repeatedly reported in all phenotypes of beta-thalassemia. Unexpectedly, in more recent studies, the concentration of total cholesterol (TC) and high- and/or low-density lipoprotein cholesterol (HDL-C and LDL-C) has been found in beta-thalassemia intermedia (TI) patients even lower than in thalassemia major, without a clear explanation of pathophysiology of these findings. This lack of information prompted us to evaluate the plasma lipids and lipoproteins pattern in the TI patients followed in our department; the data were compared with those found in hereditary spherocytosis patients. Furthermore, in both groups of patients, the erythroid bone marrow activity was evaluated, utilizing the level of soluble transferrin receptors (sTfR) in the plasma. Both groups of patients showed similar lipid abnormalities (low-TC, HDL-C and LDL-C) and the same increase of sTfR, with significantly lower hemoglobin levels in TI patients. Data analysis of our study shows that the lipid profile in TI patients is not influenced by age, sex, liver injury, hemoglobin or ferritin levels; the higher erythroid bone marrow activity with the enhanced cholesterol consumption could be the dominant mechanism implicated in the lipid abnormalities of TI patients.
Amendola
G
G
Pediatric Hematology and Oncology Section, Department of Neonatology and Pediatrics, Ospedale Umberto I, Nocera Inferiore, Salerno, Italy.
Danise
P
P
Todisco
N
N
D'Urzo
G
G
Di Palma
A
A
Di Concilio
R
R
D000293:Adolescent; D000328:Adult; D001853:Bone Marrow / Q000503:physiopathology*; D002784:Cholesterol / Q000097:blood; D005260:Female; D006801:Humans; D008055:Lipids / Q000097:blood*; D008099:Liver / Q000201:enzymology; D008297:Male; D011990:Receptors, Transferrin / Q000097:blood; D013103:Spherocytosis, Hereditary / Q000097:blood; D017086:beta-Thalassemia / Q000097:blood*
D008055:Lipids; D011990:Receptors, Transferrin; D002784:Cholesterol