Ozdogu Hakan, Boga Can, Asma Suheyl, Kozanoglu Ilknur, Gereklioglu Cigdem, Yeral Mahmut, Buyukkurt Nurhilal Turgut, Solmaz Soner, Korur Aslı, Aytan Pelin, Maytalman Erkan, Kasar Mutlu
Adana Adult Bone Marrow Transplantation Center, University Hospital of Baskent, Adana Department of Hematology Department of Family Medicine Department of Physiology Department of Immunology, Faculty of Medicine, University of Baskent, Ankara, Turkey.
Medicine (Baltimore). 2018 Feb;97(6):e9844. doi: 10.1097/MD.0000000000009844.
The Eastern Mediterranean is among the regions where sickle cell disease (SCD) is common. The morbidity and mortality of this disease can be postponed to adulthood through therapies implemented in childhood. The present study focuses on the organ damage-reducing effects of the Baskent Sickle Cell Medical Care Development Program (BASCARE), which was developed by a team who lives in this region and has approximately 25 years of experience. The deliverables of the program included the development of an electronic health recording system (PRANA) and electronic vaccination system; the use of low citrate infusion in routine prophylactic automatic erythrocyte exchange (ARCE) programs including pregnant women; the use of leukocyte-filtered and irradiated blood for transfusion; the use of magnetic resonance imaging methods (T2) for the management of transfusion-related hemosiderosis; and the implementation of an allogeneic hematopoietic stem cell transplantation protocol for adult patients. The sample was composed of 376 study subjects and 249 control subjects. The hospital's Data Management System and the central population operating system were used for data collection. BASCARE enabled better analysis and interpretation of complication and mortality data. Vaccination rates against influenza and pneumococcal disease improved (21.5% vs 50.8% and 21.5% vs 49.2%, respectively). Effective and safe ARCE with low citrate infusion were maintained in 352 subjects (1003 procedures). Maternal and fetal mortality was prevented in 35 consecutive pregnant patients with ARCE. Chelating therapy rates reduced from 6.7% to 5%. Successful outcomes could be obtained in all 13 adult patients who underwent allogeneic peripheral stem cell transplantation from a fully matched, related donor. No patients died by day 100 or after the first year. Cure could be achieved without graft loss, grades III to IV acute graft versus host disease, extensive chronic graft versus host disease, or other major complications. The BASCARE program significantly improved patient care and thereby prolonged the life span of SCD patients (42 ± 13 years vs 29 ± 7 years, P < .001). We may recommend using such individualized programs in centers that provide health care for patients with SCD, in accordance with holistic approach due to the benign nature but malignant course of the disease.
东地中海地区是镰状细胞病（SCD）常见的地区之一。通过儿童期实施的治疗，这种疾病的发病率和死亡率可以推迟到成年期。本研究聚焦于由该地区一支拥有约25年经验的团队开发的巴斯肯镰状细胞医疗护理发展项目（BASCARE）对减少器官损伤的作用。该项目的成果包括开发电子健康记录系统（PRANA）和电子疫苗接种系统；在包括孕妇在内的常规预防性自动红细胞置换（ARCE）项目中使用低枸橼酸盐输注；使用白细胞过滤和辐照血液进行输血；使用磁共振成像方法（T2）管理输血相关的含铁血黄素沉着症；以及为成年患者实施异基因造血干细胞移植方案。样本由376名研究对象和249名对照对象组成。数据收集使用了医院的数据管理系统和中央人口操作系统。BASCARE能够更好地分析和解释并发症及死亡率数据。流感和肺炎球菌疾病的疫苗接种率有所提高（分别从21.5%提高到50.8%和从21.5%提高到49.2%）。352名受试者（1003次操作）维持了使用低枸橼酸盐输注的有效且安全的ARCE。35名连续接受ARCE的孕妇预防了母婴死亡。螯合治疗率从6.7%降至5%。所有13名接受来自完全匹配的相关供体的异基因外周干细胞移植的成年患者均获得成功结果。在第100天或第一年后无患者死亡。可实现治愈，且无移植物丢失、III至IV级急性移植物抗宿主病、广泛的慢性移植物抗宿主病或其他主要并发症。BASCARE项目显著改善了患者护理，从而延长了SCD患者的寿命（42±13岁对29±7岁，P<0.001）。鉴于该疾病性质良性但病程恶性，我们建议在为SCD患者提供医疗服务的中心根据整体方法使用此类个体化项目。
