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重型地中海贫血患者外周血单核细胞的吞噬溶酶体融合减少。

Decreased phagolysosomal fusion of peripheral blood monocytes from patients with thalassemia major.

作者信息

Pittis M G, Estevez M E, Diez R A, de Miani S A, Sen L

机构信息

Instituto de Investigaciones Hematológicas Mariano R. Castex, Academia Nacional de Medicina, Buenos Aires, Argentina.

出版信息

Acta Haematol. 1994;92(2):66-70. doi: 10.1159/000204177.

Abstract

We evaluated the phagolysosomal fusion of peripheral blood monocytes from 15 patients with thalassemia major and 10 thalassemia major carriers using a cytomorphological method with acridine orange as fusion marker. The monocyte phagolysosomal fusion of thalassemic patients was decreased (49.6 +/- 8.6%, mean +/- SD) and differed significantly (p < 0.05) from those of carriers and normal controls (65.7 +/- 11.4% and 74.6 +/- 5.7%, respectively). In vitro deferoxamine partially improved monocyte phagolysosomal fusion of patients with thalassemia major, and did not affect monocyte function in carriers and healthy subjects. Furthermore, in vitro addition of ferrous sulfate decreased normal phagolysosomal fusion. We conclude that the monocyte phagolysosomal fusion dysfunction of thalassemic patients could be related to iron overload.

摘要

我们采用以吖啶橙作为融合标记的细胞形态学方法,评估了15例重型地中海贫血患者和10例重型地中海贫血携带者外周血单核细胞的吞噬溶酶体融合情况。重型地中海贫血患者的单核细胞吞噬溶酶体融合率降低(49.6±8.6%,平均值±标准差),与携带者和正常对照组(分别为65.7±11.4%和74.6±5.7%)相比有显著差异(p<0.05)。体外去铁胺可部分改善重型地中海贫血患者的单核细胞吞噬溶酶体融合,且不影响携带者和健康受试者的单核细胞功能。此外,体外添加硫酸亚铁会降低正常的吞噬溶酶体融合。我们得出结论,重型地中海贫血患者的单核细胞吞噬溶酶体融合功能障碍可能与铁过载有关。

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