Matzner Y, Goldfarb A, Abrahamov A, Drexler R, Friedberg A, Rachmilewitz E A
Department of Haematology, Hadassah University Hospitals, Jerusalem, Israel.
Br J Haematol. 1993 Sep;85(1):153-8. doi: 10.1111/j.1365-2141.1993.tb08659.x.
Random and directed migration, O2- production, degranulation and adhesion were studied in neutrophils obtained from patients with homozygous beta-thalassaemia and iron overload, in the presence or absence of thalassaemic serum. The only significant defect found was an impairment in directed chemotaxis, further depressed after addition of thalassaemic serum. The chemotactic defect was encountered in all the patients that have suffered from pyogenic infections except one, and was not correlated with the severity of the iron overload. It is suggested that the described neutrophil migration impairment may contribute to the tendency towards infection in certain patients with homozygous beta-thalassaemia.
在有或无地中海贫血血清存在的情况下，对从纯合子β地中海贫血和铁过载患者中获取的中性粒细胞的随机和定向迁移、O₂生成、脱颗粒和黏附进行了研究。发现的唯一显著缺陷是定向趋化性受损，加入地中海贫血血清后进一步降低。除一名患者外，所有患有化脓性感染的患者均出现趋化缺陷，且与铁过载的严重程度无关。提示所描述的中性粒细胞迁移受损可能导致某些纯合子β地中海贫血患者易发生感染。
