C3 Polymorphism in Beta-Thalassemia

Germenis, Anastasios E.; Kalantzi, Maria; Karamerou, A.; Politi, Cristina; Fertakis, A

doi:10.1159/000206507

Acta Haematol

1984

DOI: 10.1159/000206507

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C3 Polymorphism in Beta-Thalassemia

Anastasios E. Germenis

Maria Kalantzi

A. Karamerou

et al.

Abstract: The distribution of phenotypes and gene frequencies of the third component of complement (C3) were studied in 106 β-thalassemic patients and in 112 carriers of the β-thalassemia trait. A statistically significant association was found between the C3F gene and homozygous β-thalassemia. It can be suggested that this association may be related with the high incidence of infections encountered in these patients.

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1986

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Progressive dysfunction of monocytes associated with iron overload and age in patients with thalassemia major

Ballart¹,

Estévez²,

Sen³

et al. 1986

Blood

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We evaluated phagocytic and lytic activities of peripheral blood monocytes (PBMo) from patients with thalassemia major (ThP) using C pseudotropicalis as the target. PBMo from ThP showed decreased lytic activity (P less than .001), whereas the phagocytic activity did not differ from that of the controls. Significant inverse correlations were found between lytic activity of PBMo and age of patients (r2 = .47; P less than .01) and also between lytic activity and serum ferritin levels (r2 = .65; P less than .001). No association was found between lytic activity and other variables (blood transfusion regimens, therapy with desferrioxamine, liver damage, and the presence of sHBAg). Splenectomy showed no positive effect on PBMo functions from ThP. Our results suggest that PBMo from ThP have an intracellular defect in their microbicidal mechanisms associated with iron overload. This cell dysfunction could be responsible, at least in part, for the increased susceptibility to infections reported in ThP.

show abstract

Progressive dysfunction of monocytes associated with iron overload and age in patients with thalassemia major

Ballart¹,

Estévez²,

Sen³

et al. 1986

Blood

View full text Add to dashboard Cite

show abstract

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C3 Polymorphism in Beta-Thalassemia

Cited by 1 publication

References 11 publications

Progressive dysfunction of monocytes associated with iron overload and age in patients with thalassemia major

Progressive dysfunction of monocytes associated with iron overload and age in patients with thalassemia major

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