纯合子镰状细胞病中的红细胞变形性和聚集性。

Erythrocyte deformability and aggregation in homozygous sickle cell disease.

作者信息

Vayá Amparo, Collado Susana, Dasí Maria Angeles, Pérez Maria Luz, Hernandez Jose Luis, Barragán Eva

机构信息

Haemorheology and Haemostasis Unit, Service of Clinical Pathology, La Fe University Hospital, Valencia, Spain.

Pediatry Service, La Fe University Hospital, Valencia, Spain.

出版信息

Clin Hemorheol Microcirc. 2014;58(4):497-505. doi: 10.3233/CH-131717.

DOI:10.3233/CH-131717

PMID:23603322

Abstract

Rheological properties of homozygous sickle cell anaemia (SCA) show marked heterogeneity, which may be explained in part by the concomitance of alpha genotypes or beta haplotypes, along with hydroxurea (HU) treatment. To further clarify this issue, in 11 homozygous patients with SCA in the steady state and in 16 healthy controls, we analysed erythrocyte deformability (ED) in a Rheodyn SSD by means of the Elongation Index (EI) at 12, 30 and 60 Pa, and erythrocyte aggregation at stasis (EA0) and at 3 sec-1 (EA1) in a Myrenne aggregometer along with fibrinogen, biochemical and haematological parameters. When compared with controls, homozygous (SS) patients showed a lower EI at all the shear stresses tested (p < 0.01) and higher EA0 (p < 0.014), but not higher EA1 (p = 0.076). Fibrinogen did not show statistical differences (p = 0.642). In the Spearman's correlation IE60 correlated inversely with Hb S (p < 0.05) and directly with MCV, MCH and Hb F levels (p < 0.01). EA0 correlated inversely with MCV, MCH, Hb F (p < 0.01) and directly with Hb S (p < 0.05). HU treatment improved EI and EA0, but not EA1. This paradoxical behaviour of HU on erythrocyte aggregation merits further research to be clarified.

摘要

纯合子镰状细胞贫血（SCA）的流变学特性表现出显著的异质性，这部分可能由α基因型或β单倍型的并存以及羟基脲（HU）治疗来解释。为了进一步阐明这个问题，我们对11例处于稳定状态的纯合子SCA患者和16名健康对照者进行了研究，通过在12、30和60 Pa下的伸长指数（EI），利用Rheodyn SSD分析红细胞变形性（ED），并在Myrenne血细胞聚集仪中分析静态时（EA0）和3秒-1时（EA1）的红细胞聚集情况，同时检测纤维蛋白原、生化和血液学参数。与对照组相比，纯合子（SS）患者在所有测试的剪切应力下EI均较低（p < 0.01），EA0较高（p < 0.014），但EA1不高（p = 0.076）。纤维蛋白原无统计学差异（p = 0.642）。在Spearman相关性分析中，IE60与Hb S呈负相关（p < 0.05），与MCV、MCH和Hb F水平呈正相关（p < 0.01）。EA0与MCV、MCH、Hb F呈负相关（p < 0.01），与Hb S呈正相关（p < 0.05）。HU治疗改善了EI和EA0，但未改善EA1。HU对红细胞聚集的这种矛盾行为值得进一步研究以阐明。