在资源有限的情况下骨髓移植治疗镰状细胞病的挑战

C. Okolo, A. Olayanju
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引用次数: 0

摘要

镰状细胞疾病(SCD)是由于父母双方都是血红蛋白携带者而导致血红蛋白(SS)的一组遗传性疾病,可引起贫血、感染、疼痛、中风、阴茎勃起功能障碍、隔离危机、多器官功能障碍等。治疗镰状细胞病有几种方法,但最好的方法,被认为是治疗该病的金标准,是成功的骨髓移植造血干细胞(HSCT)。骨髓(BM)、外周血(PBSC)和脐带血(UCB)中含有丰富的干细胞。为了进行良好的骨髓移植,没有移植排斥反应,实验室在移植的各个阶段起着至关重要的作用,特别是在移植的各个阶段,包括初始阶段,移植期间和后续测试,以确保对新移植的耐受性和嵌合性测试。有各种类型的移植,包括自体,异体(兄弟姐妹/非亲属供体),父母或亲属等。各种移植前检查包括:镰状细胞试验、高效液相色谱仪(HPLC)、遗传学研究、红细胞指数Hb - 6-9 gm/dL、外周涂片、红细胞计数、红细胞血沉、血沉、骨髓分析。移植后检查包括移植分析、全血细胞计数、肾功能、肝功能、胆固醇、艾滋病毒、乙型肝炎、丙型肝炎、其他传染病研究(地方性检测)、尿液分析等。在尼日利亚这样资源有限的国家,造血移植面临着巨大的挑战,包括:与供体对患者干细胞移植相关的严重疾病的危险、缺乏适当的供体、移植物抗宿主病(GVHD)、人力资源不足、设备不足、服务成本高、腐败、缺乏政治意愿、领导缺乏协调、缺乏战略规划、政策不一致、缺乏合格人员、卫生保健管理不善、卫生工作者之间的利益冲突。骨髓移植的费用对尼日利亚人来说是非常高昂的,政府需要在资助部分费用和减轻镰状细胞的痛苦方面发挥至关重要的作用。有效实施国家健康保险计划和解决腐败问题是补贴成本的最有效方式。主要由妇幼保健专业人员倡导的适当宣传运动将大大有助于提高对脐带血捐献的认识和接受程度。对医务人员的培训和再培训再强调也不为过。
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Challenges of bone marrow transplant for sickle cell disease in resource limited setting
Sickle cell disorders (SCD) are a group of inherited disorders that results from both parent being carriers, haemoglobin AS resulting in haemoglobin (SS), causing anemia, infections, pain, stroke, priapism, sequenstration crisis, multiple organ dysfunctions etc. There are several ways of managing sickle cell disorder but the best way, which is considered a gold standard cure for the disease is a successful bone marrow transplant of haematopoietic stem cells (HSCT). Bone marrow (BM), Peripheral blood (PBSC) and Umbilical cord blood (UCB) are rich in stem cells. In order to have a good bone marrow transplant, without graft rejection, the laboratory plays a vital role especially in compatibility testing of donor and recipients at the various stages of the transplant, which includes initial stage, during the transplant and follow up testing to ensure tolerance to the new graft and testing for chimerism. There are various types of transplant which include Autologous, Allogeneic (Sibling/ Unrelated Donor), Parent or relative, etc. The various pre transplant tests includes: Sickling test, High Performance Liquid Chromatograph (HPLC), Genetic studies, RBC Indices Hb – 6-9 gm/dL, Peripheral Smear, Retic count Reticulocytosis, ESR, Bone marrow analysis. Post-transplant test Includes Engraftment Analysis, Complete Blood counts, kidney function, liver function, Cholesterol, HIV, Hepatitis B, Hepatitis C, additional infectious studies (Endemic testing), Urinalysis etc. Haemopoietic transplantation challenges in a resource limited settings like Nigeria are enormous and they includes: Danger of serious illness associated with donor-to-patient stem cell transplant, lack of appropriate donors, Graft-versus-host disease (GVHD), Inadequate Human resource, Inadequate equipped facility, cost of the service, Corruption, Lack of political will, Leadership incompétence, Lack of strategic planing, Policy inconsistency, Lack of qualified personnel, Poor healthcare administration, Conflict of interest among health workers. The cost of Bone Marrow Transplantation is highly exorbitant to Nigerians and government need to play vital role towards funding part of the cost and alleviating the pain of sickle cell. Effective implementation of National Health Insurance Scheme and address corruption are the most efficient ways the cost would be subsidized. Appropriate information campaigns largely championed by maternal and child health-care professionals would significantly contribute to raising the level of awareness and acceptance of Cord Blood donation. Training and re training of medical personnel cannot be over emphasized.
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