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[多次输血的地中海贫血患者中性粒细胞趋化功能障碍]

[Neutrophil chemotactic dysfunction in multitransfused thalassemia patients].

作者信息

Palacios M F, Testoni R A, Ballart I J, de Miani S A, Díez R A, Estevez M E, Sen L

机构信息

División Inmunología Oncológica, IIHEMA, Academia Nacional de Medicina, Buenos Aires, Argentina.

出版信息

Sangre (Barc). 1993 Aug;38(4):295-9.

PMID:8235944
Abstract

PURPOSE

To evaluate the chemotactic capability of neutrophils in thalassaemic patients under poly-transfusion regimens and in thalassaemia carriers.

PATIENTS AND METHODS

Twenty-one patients in multi-transfusion regimen diagnosed in the Ricardo Gutiérrez Children Hospital were studied. Of them, 17 had thalassaemia major, 3 S/beta thalassaemia and one sickle-cell anaemia. Twenty-one normal subjects comprised a control group. Chemotaxis was evaluated by two methods, namely, migration under agarose layer and in microchemotaxis chamber under stimulation with N-formyl-methionyl-n-phenylalanine at optimal concentrations of 10(-5) M and 10(-6) M, respectively.

RESULTS

In thalassaemia major patients, directed mobility of neutrophil assessed by both methods was significantly decreased with regard to the normal controls, whereas random mobility was preserved. The four patients under poly-transfusion who had not thalassaemia major showed the same neutrophil defect. On the contrary, chemotaxis and random mobility of the neutrophils from thalassaemia carriers (thalassaemia minor) were similar to those of the normal controls.

CONCLUSIONS

These results suggest that the defect found in the patients might be caused by transfusion overload.

摘要

目的

评估接受多次输血治疗的地中海贫血患者及地中海贫血携带者中性粒细胞的趋化能力。

患者与方法

对里卡多·古铁雷斯儿童医院诊断为接受多次输血治疗的21例患者进行研究。其中,17例为重型地中海贫血,3例为S/β地中海贫血,1例为镰状细胞贫血。21名正常受试者作为对照组。趋化性通过两种方法进行评估,即分别在最佳浓度为10⁻⁵ M和10⁻⁶ M的N-甲酰甲硫氨酰-n-苯丙氨酸刺激下,在琼脂糖层下迁移和在微量趋化性小室中迁移。

结果

重型地中海贫血患者中,通过两种方法评估的中性粒细胞定向移动性相对于正常对照组均显著降低,而随机移动性得以保留。4例接受多次输血但非重型地中海贫血的患者表现出相同的中性粒细胞缺陷。相反,地中海贫血携带者(轻型地中海贫血)中性粒细胞的趋化性和随机移动性与正常对照组相似。

结论

这些结果表明,患者中发现的缺陷可能是由输血过量引起的。

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