Seck Moussa, Ba Oumou, Faye Blaise Felix, Touré Sokhna Aissatou, Guèye Youssou Bamar, Dieng Nata, Sall Abibatou, Gadji Macoura, Touré Awa Oumar, Diop Saliou
Hematology Department Cheikh Anta Diop University Dakar Senegal.
National Blood Transfusion Center Clinical Hematology Dakar Senegal.
EJHaem. 2021 Oct 4;2(4):711-715. doi: 10.1002/jha2.273. eCollection 2021 Nov.
Homozygous sickle cell disease (HSCD) is characterized by multiorgan morbidity and an increased risk of early death. We aim to describe the mortality rate, causes, and risk factors of death in HSCD between 2011 and 2020. We conducted a retrospective study with a duration of 10 years in the cohort of 2348 HSCD patients. The mortality rate was determined by reporting the number of deaths to the total number of patients followed in the year. Sociodemographic, clinical, biological data and causes of death were studied. Death risk factors were determined by a bivariate analysis comparing deceased and living HSCD patients. The mean age of death was 26 years (3-52). The sex ratio was 1.2. The mortality rate was 2.76%. The death rate was high in 2011 (3.2%) and low in 2020 (0.17%). We observed a significant reduction of mortality of 94.6%. Most of the common causes of death were acute anemia (40%), acute chest syndrome (24.6%), and infections (20%). Risk factors of death were age, vaso-occlusive crises ≥3, acute chest syndrome, blood transfusion, and chronic complications. Mortality among HSCD has significantly decreased over the past 10 years in Senegal, and the main causes of death were acute anemia, acute chest syndrome, and infections.
纯合子镰状细胞病（HSCD）的特征是多器官发病以及早期死亡风险增加。我们旨在描述2011年至2020年间HSCD的死亡率、死亡原因和死亡风险因素。我们对2348例HSCD患者队列进行了为期10年的回顾性研究。死亡率通过报告当年死亡人数与随访患者总数来确定。研究了社会人口统计学、临床、生物学数据及死亡原因。通过对已故和在世的HSCD患者进行双变量分析来确定死亡风险因素。平均死亡年龄为26岁（3至52岁）。男女比例为1.2。死亡率为2.76%。2011年死亡率较高（3.2%），2020年较低（0.17%）。我们观察到死亡率显著降低了94.6%。大多数常见死亡原因是急性贫血（40%）、急性胸综合征（24.6%）和感染（20%）。死亡风险因素包括年龄、血管闭塞性危机≥3次、急性胸综合征、输血和慢性并发症。在塞内加尔，过去10年中HSCD的死亡率显著下降，主要死亡原因是急性贫血、急性胸综合征和感染。
