Garnier Yohann, Ferdinand Séverine, Etienne-Julan Maryse, Elana Gisèle, Petras Marie, Doumdo Lydia, Tressières Benoit, Lalanne-Mistrih Marie-Laure, Hardy-Dessources Marie-Dominique, Connes Philippe, Romana Marc
Unité Biologie Intégrée du Globule Rouge, Université des Antilles, Inserm 1134, laboratoire d'Excellence GR-Ex, Paris, France.
Unité Transversale de la Drépanocytose, CHU de Pointe-à-Pitre, Pointe-à-Pitre, Guadeloupe, France.
PLoS One. 2017 May 10;12(5):e0177397. doi: 10.1371/journal.pone.0177397. eCollection 2017.
Sickle cell anemia (SCA) and hemoglobin SC (HbSC) disease are the two most common forms of sickle cell disease (SCD), a frequent hemoglobinopathy which exhibits a highly variable clinical course. Although high levels of microparticles (MPs) have been consistently reported in SCA and evidence of their harmful impact on the SCA complication occurrences have been provided, no data on MP pattern in HbSC patients has been reported so far. In this study, we determined and compared the MP patterns of 84 HbSC and 96 SCA children, all at steady-state, using flow cytometry. Most of circulating MPs were derived from platelets (PLTs) and red blood cells (RBCs) in the two SCD syndromes. Moreover, we showed that HbSC patients exhibited lower blood concentration of total MPs compared to SCA patients, resulting mainly from a decrease of MP levels originated from RBCs and to a lesser extent from PLTs. We did not detect any association between blood MP concentrations and the occurrence of painful vaso-occlusive crises, acute chest syndrome and pulmonary hypertension in both patient groups. We also demonstrated for the first time, that whatever the considered genotype, RBC-derived MPs exhibited higher externalized phosphatidylserine level and were larger than PLT-derived MPs.
镰状细胞贫血(SCA)和血红蛋白SC(HbSC)病是镰状细胞病(SCD)最常见的两种形式,SCD是一种常见的血红蛋白病,临床病程高度可变。尽管在SCA中一直有关于高水平微粒(MPs)的报道,并且已经提供了它们对SCA并发症发生有有害影响的证据,但迄今为止尚未有关于HbSC患者MP模式的数据报道。在本研究中,我们使用流式细胞术测定并比较了84例处于稳定状态的HbSC儿童和96例SCA儿童的MP模式。在这两种SCD综合征中,大多数循环MPs来源于血小板(PLTs)和红细胞(RBCs)。此外,我们发现与SCA患者相比,HbSC患者血液中总MPs浓度较低,这主要是由于源自RBCs的MP水平降低,而源自PLTs的MP水平降低程度较小。我们未在两组患者中检测到血液MP浓度与疼痛性血管闭塞危象、急性胸综合征和肺动脉高压的发生之间存在任何关联。我们还首次证明,无论考虑何种基因型,源自RBCs的MPs均表现出更高的外化磷脂酰丝氨酸水平,并且比源自PLTs的MPs更大。