Seck Moussa, Dabo Maureen Adéniké, Bousso Elimane Seydi, Keita Mohamed, Touré Sokhna Aïssatou, Guèye Sérigne Mourtalla, Faye Blaise Félix, Dieng Fatma, Diop Saliou
Hematology Department, Cheikh Anta Diop University of Dakar, Dakar, Senegal.
National Blood Transfusion Center of Dakar, BP 5002, Fann, Dakar, Senegal.
Adv Hematol. 2024 Feb 6;2024:7501577. doi: 10.1155/2024/7501577. eCollection 2024.
The aim of this study was to describe the morbidity and mortality of homozygous sickle cell disease after the age of 40.
This was a cohort study of 209 patients followed from 1994 to 2022. All hemoglobin electrophoresis-confirmed SS sickle cell patients over 40 years were included. A descriptive study of epidemiological, diagnostic, therapeutic, and evolutionary data was used to assess morbidity and mortality.
Sex ratio (M/F) was 0.6. Median age was 47 (41-75). According to morbidity, 95.1% had less than 3 vaso-occlusive crises/year. Acute anemia was the most frequent complication (52.63%). Chronic complications were noted in 32.5%. At diagnosis, mean hemoglobin was 8.1 g/dl ± 1.9, HbS was 86.5 ± 10, and HbF was 9.4 ± 7.6. Number of patients transfused was 66%. We noted that 8.1% of patients died, 29.2% were lost to follow-up, and 62.7% were still being followed up. The risk factors identified for death were geographical origin, comorbidity, high HbS, low HbF, and thrombocytosis.
This study shows that homozygous SCD is increasingly becoming an adult disease and that it can be carried into old age in Africa. Advanced age over 40 is marked by an upsurge in chronic complications, making it essential to set up a screening program and to organize multidisciplinary follow-up.
本研究旨在描述40岁以后纯合子镰状细胞病的发病率和死亡率。
这是一项对209例患者进行的队列研究，随访时间为1994年至2022年。纳入所有血红蛋白电泳确诊的40岁以上SS型镰状细胞病患者。采用对流行病学、诊断、治疗和病情演变数据的描述性研究来评估发病率和死亡率。
性别比（男/女）为0.6。中位年龄为47岁（41 - 75岁）。根据发病率，95.1%的患者每年血管闭塞性危象少于3次。急性贫血是最常见的并发症（52.63%）。32.5%的患者出现慢性并发症。诊断时，平均血红蛋白为8.1 g/dl ± 1.9，血红蛋白S为86.5 ± 10，血红蛋白F为9.4 ± 7.6。接受输血的患者比例为66%。我们注意到8.1%的患者死亡，29.2%的患者失访，62.7%的患者仍在随访中。确定的死亡危险因素为地理来源、合并症、高血红蛋白S、低血红蛋白F和血小板增多症。
本研究表明，纯合子镰状细胞病在非洲正日益成为一种成人疾病，并且可以延续至老年。40岁以上的高龄患者慢性并发症激增，因此必须建立筛查项目并组织多学科随访。
