van Zuuren Esther J, Fedorowicz Zbys
Department of Dermatology, Leiden University Medical Centre , Leiden , The Netherlands .
Hemoglobin. 2014;38(3):221-3. doi: 10.3109/03630269.2014.911748.
We summarize a Cochrane systematic review that was conducted to assess the effects of low-molecular-weight heparins (LMWH) for managing vasoocclusive crises (VOC) in people with sickle cell disease. Sickle cell disease is one of the most common and severe genetic disorders in the world. It can be divided into three broadly distinct clinical phenotypes characterized by either hemolysis, pain syndromes or organ damage. Pain is the most prominent symptom of vasoocclusion, and hypercoagulability is a well-established pathogenic phenomenon in people with sickle cell disease. Searches included the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register, abstract books of conference proceedings and several online trials registries (December 2012). One study (with an overall unclear to high risk of bias) comprising 253 participants was included. This study provided limited data, but concluded that tinzaparin resulted in a more rapid resolution of pain, and in a statistically significant lower number of hospitalization days compared to a placebo. Two minor bleeding events were reported as adverse events in the tinzaparin group. Based on the results from this single study, there is incomplete evidence to either support or refute the effectiveness of LMWH in people with sickle cell disease.
我们总结了一项Cochrane系统评价,该评价旨在评估低分子量肝素(LMWH)对镰状细胞病患者血管闭塞性危象(VOC)的治疗效果。镰状细胞病是世界上最常见且最严重的遗传性疾病之一。它可大致分为三种不同的临床表型,其特征分别为溶血、疼痛综合征或器官损伤。疼痛是血管闭塞最突出的症状,而高凝状态是镰状细胞病患者中公认的致病现象。检索范围包括Cochrane囊性纤维化和遗传性疾病血红蛋白病试验注册库、会议论文摘要集以及多个在线试验注册库(2012年12月)。纳入了一项研究(总体偏倚风险不明确至高),该研究包含253名参与者。这项研究提供的数据有限,但得出结论,与安慰剂相比,替扎肝素能使疼痛缓解更快,且住院天数在统计学上显著减少。替扎肝素组报告了两例轻微出血事件作为不良事件。基于这项单一研究的结果,目前尚无充分证据支持或反驳LMWH对镰状细胞病患者的有效性。