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重型地中海贫血多次输血患儿铁相关的细胞介导免疫紊乱

Iron-related disturbances of cell-mediated immunity in multitransfused children with thalassemia major.

作者信息

Pardalos G, Kanakoudi-Tsakalidis F, Malaka-Zafiriu M, Tsantali H, Athanasiou-Metaxa M, Kallinikos G, Papaevangelou G

机构信息

First Department of Pediatrics, Aristotle University of Thessaloniki, Greece.

出版信息

Clin Exp Immunol. 1987 Apr;68(1):138-45.

Abstract

Immunological abnormalities have been observed in many haemophiliacs receiving clotting factor concentrates. To determine whether similar changes also occur after repeated blood transfusions we estimated T cell subsets and cutaneous delayed hypersensitivity (CDH) in 50 multitransfused children with beta-thalassemia major (beta-TM). All patients were also tested for anti-HTLV-III/LAV antibodies. A diminished percentage of T lymphocytes (E-rosettes, T3+), and T4+ cells and a low T4/T8 ratio was found in patients as compared to age and sex matched controls (P less than 0.001). Negative CDH tests to specific antigens (Multi-test) were also found in a significantly larger proportion of beta-TM children (P less than 0.01). Antibodies against HTLV-III/LAV were negative in all patients. Decreased T4/T8 ratio in beta-TM children was primarily due to a reduction of T4+ cells and was inversely correlated to the patients' age, number of units of transfused blood (P less than 0.05) and especially to ferritin serum levels and annual iron balance (P less than 0.001). These findings indicate that immunological abnormalities in beta-TM patients appear to be acquired, transfusion-associated and related to iron load which depends on the appropriate chelation therapy.

摘要

在许多接受凝血因子浓缩物治疗的血友病患者中已观察到免疫异常。为了确定反复输血后是否也会出现类似变化,我们对50例多次输血的重型β地中海贫血(β-TM)患儿的T细胞亚群和皮肤迟发型超敏反应(CDH)进行了评估。所有患者还检测了抗HTLV-III/LAV抗体。与年龄和性别匹配的对照组相比,患者的T淋巴细胞(E花环、T3+)和T4+细胞百分比降低,T4/T8比值较低(P<0.001)。在β-TM患儿中,对特定抗原(多项检测)的CDH试验阴性的比例也明显更高(P<0.01)。所有患者的抗HTLV-III/LAV抗体均为阴性。β-TM患儿T4/T8比值降低主要是由于T4+细胞减少,且与患者年龄、输血量(P<0.05)尤其是血清铁蛋白水平和年度铁平衡呈负相关(P<0.001)。这些发现表明,β-TM患者的免疫异常似乎是后天获得的、与输血相关的,且与铁负荷有关,而铁负荷取决于适当的螯合疗法。

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本文引用的文献

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