title: Increased serum levels of macrophage colony-stimulating factor (M-CSF) in alpha- and beta-thalassaemia syndromes: correlation with anaemia and monocyte activation.
abstract: Serum levels of M-CSF were determined by an ELISA method in 29 and 34 patients with HbH disease (alpha 1/alpha 2 or alpha 2/HbCS) or beta zero-thal/HbE, respectively, in 28 haematologically normal subjects and in five patients with anaemia due to iron deficiency or myelodysplasia. In HbH disease and beta zero-thal/HbE, M-CSF concentrations were significantly higher than those in the normal subjects [986 +/- 138 and 1385 +/- 133, respectively, vs. 500 +/- 33 pg/ml (mean +/- SEM); p < 0.01, and p < 0.001, respectively]. By contrast, in patients with anaemia due to iron deficiency, M-CSF levels were within the normal range. In HbH disease and in beta zero-thal/HbE, M-CSF levels correlated inversely with mean basal Hb values (r = -0.39, p = 0.05 and r = -0.60, p < 0.001, respectively). In addition, in some of the HbH and beta zero-thal/HbE patients, monocyte ADCC activities towards red cells were tested and found to be approximately twice as high as those in normal controls [38.3 +/- 5.7 and 30.7 +/- 4.6 vs. 17.8 +/- 1.8% specific lysis (mean +/- SEM), respectively; p < 0.01 and p < 0.02, respectively]. When thalassaemic patients and normal controls were considered together there was a significant correlation between M-CSF levels and monocyte ADCC activities (r = 0.51, p < 0.02). The results suggest that in HbH disease and in beta zero-thal/HbE, raised serum M-CSF contributes to the anaemia by enhancing the effector function of mononuclear phagocytes towards red cells.
authors.affiliation: Department of Hematology, Imperial College School of Medicine at St. Mary's, London. e.wiener@ic.ac.uk
authors.forename: E
authors.initials: E
authors.lastname: Wiener
authors.forename: W
authors.initials: W
authors.lastname: Wanachiwanawin
authors.forename: S
authors.initials: S
authors.lastname: Chinprasertsuk
authors.forename: U
authors.initials: U
authors.lastname: Siripanyaphinyo
authors.forename: F
authors.initials: F
authors.lastname: Mawas
authors.forename: S
authors.initials: S
authors.lastname: Fucharoen
authors.forename: S N
authors.initials: SN
authors.lastname: Wickramasinghe
mesh_terms: D000293:Adolescent; D000328:Adult; D015415:Biomarkers; D002648:Child; D005260:Female; D006801:Humans; D007501:Iron / Q000378:metabolism; D016173:Macrophage Colony-Stimulating Factor / Q000097:blood*; D008297:Male; D008875:Middle Aged; D009000:Monocytes / Q000473:pathology*; D010587:Phagocytosis; D017085:alpha-Thalassemia / Q000097:blood* / Q000473:pathology; D017086:beta-Thalassemia / Q000097:blood* / Q000473:pathology
chemical_list: D015415:Biomarkers; D016173:Macrophage Colony-Stimulating Factor; D007501:Iron