Inserm U665, CHU de Pointe à Pitre, Hôpital Ricou, 97159 Pointe-à-Pitre, Guadeloupe.
Haematologica. 2012 Nov;97(11):1641-7. doi: 10.3324/haematol.2012.066670. Epub 2012 Jun 11.
Little is known about the effects of blood rheology on the occurrence of acute chest syndrome and painful vaso-occlusive crises in children with sickle cell anemia and hemoglobin SC disease.
To address this issue, steady-state hemorheological profiles (blood viscosity, red blood cell deformability, aggregation properties) and hematologic parameters were assessed in 44 children with sickle cell anemia and 49 children with hemoglobin SC disease (8-16 years old) followed since birth. Clinical charts were retrospectively reviewed to determine prior acute chest syndrome or vaso-occlusive episodes, and rates of these complications were calculated.
Multivariate analysis revealed that: 1) a higher steady-state blood viscosity was associated with a higher rate of vaso-occlusive crises in children with sickle cell anemia, but not in children with hemoglobin SC disease; 2) a higher steady-state red blood cell disaggregation threshold was associated with previous history of acute chest syndrome in children with hemoglobin SC disease and boys with sickle cell anemia.
Our results indicate for the first time that the red blood cell aggregation properties may play a role in the pathophysiology of acute chest syndrome in children with hemoglobin SC disease and boys with sickle cell anemia. In addition, whereas greater blood viscosity is associated with a higher rate of vaso-occlusive crises in children with sickle cell anemia, no association was found in children with hemoglobin SC disease, underscoring differences in the etiology of vaso-occlusive crises between sickle cell anemia and hemoglobin SC disease.
关于血液流变学对镰状细胞贫血和血红蛋白 SC 病患儿急性胸部综合征和疼痛性血管阻塞性危象发生的影响知之甚少。
为解决这一问题，对 44 例镰状细胞贫血患儿和 49 例血红蛋白 SC 病患儿（8-16 岁）进行了稳态血液流变学特征（血液粘度、红细胞变形性、聚集特性）和血液学参数评估。回顾性分析临床病历以确定既往急性胸部综合征或血管阻塞性发作，并计算这些并发症的发生率。
多变量分析显示：1）稳态血液粘度较高与镰状细胞贫血患儿血管阻塞性危象发生率较高相关，但与血红蛋白 SC 病患儿无关；2）稳态红细胞解聚阈值较高与血红蛋白 SC 病患儿既往有急性胸部综合征史和镰状细胞贫血男孩相关。
我们的研究结果首次表明，红细胞聚集特性可能在血红蛋白 SC 病患儿和镰状细胞贫血男孩的急性胸部综合征发病机制中起作用。此外，血液粘度增加与镰状细胞贫血患儿血管阻塞性危象发生率增加相关，但在血红蛋白 SC 病患儿中未发现相关性，这突出了镰状细胞贫血和血红蛋白 SC 病血管阻塞性危象病因的差异。
