Ballart I J, Estevez M E, Sen L, Diez R A, Giuntoli J, de Miani S A, Peñalver J
Blood. 1986 Jan;67(1):105-9.
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.
我们以假热带念珠菌为靶标，评估了重型地中海贫血患者（ThP）外周血单核细胞（PBMo）的吞噬和溶解活性。ThP患者的PBMo显示出溶解活性降低（P < 0.001），而吞噬活性与对照组无差异。发现PBMo的溶解活性与患者年龄之间存在显著负相关（r2 = 0.47；P < 0.01），并且在溶解活性与血清铁蛋白水平之间也存在显著负相关（r2 = 0.65；P < 0.001）。未发现溶解活性与其他变量（输血方案、去铁胺治疗、肝损伤和sHBAg的存在）之间存在关联。脾切除术对ThP患者的PBMo功能没有积极影响。我们的结果表明，ThP患者的PBMo在其与铁过载相关的杀菌机制中存在细胞内缺陷。这种细胞功能障碍可能至少部分导致了ThP患者报告的感染易感性增加。
