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首页> 外文期刊>Transfusion: The Journal of the American Association of Blood Banks >How do I incorporate red cell genotyping to improve chronic transfusion therapy?
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How do I incorporate red cell genotyping to improve chronic transfusion therapy?

机译:如何掺入红细胞基因分型以改善慢性输血治疗?

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BACKGROUND Children with transfusion dependent anemia, such as sickle cell disease (SCD) and thalassemia, are at an increased risk for developing red blood cell (RBC) alloantibodies due to their lifelong need for transfusion therapy. With the advent of genotyping, extended RBC antigen typing can be incorporated into chronic transfusion therapy programs (CTTPs) to improve patient care and provide antigen matched blood for this population of patients. STUDY DESIGN AND METHODS The hospital, blood center (BC), and hematology clinic caring for children requiring long‐term transfusion support developed a CTTP. Genotyping was performed at entry to determine patient RBC antigen type. Limited versus extended antigen matching of transfusions was provided based on known RBC antibodies. RESULTS Fifty patients with the following disorders were enrolled: 20 with SCD, 23 with thalassemia, and 7 with other disorders. At enrollment, nine (18%) had RBC alloantibodies, including six (30%) of patients with SCD and three (13%) with thalassemia. Two children developed antibodies after enrollment; one warm autoantibody following limited “CEK” matched RBCs and one patient with a hemizygous variant RHD allele developed anti‐D. Six (30%) patients with SCD had variant RHCE alleles; two had homozygous variant alleles and four had a variant present along with a wild type allele. CONCLUSION We demonstrate how a CTTP can be developed in a community hospital through collaboration with the blood supplier, hospital, and clinical care team. A model of incorporating RBC genotyping informs risk for alloimmunization and allows consideration of transfusion strategy for providing prophylactic antigen matched blood.
机译:背景技术患有输血依赖性贫血(例如镰状细胞疾病(SCD)和地中海贫血,由于它们的终身需要转染治疗而导致红细胞(RBC)AlloAlibodies的风险增加。随着基因分型的出现,扩展的RBC抗原打字可以掺入慢性输血治疗方案(CTTP)中,以改善患者护理,并为本患者提供抗原匹配血液。研究和方法医院,血液中心(BC)和血液学诊所,用于需要长期输血支持的儿童发出CTTP。在进入中进行基因分型以确定患者RBC抗原类型。基于已知的RBC抗体提供了有限的输血抗抗原匹配。结果50例患有以下障碍的患者注册:20例SCD,23例,23例,患有Thalassemia,7例与其他疾病。在注册时,九(18%)有RBC AlloAlibodies,包括六(30%)的SCD患者和三种(13%)的患者。两个孩子在注册后开发了抗体;在有限的“CEK”匹配的RBC和一个患有患有嗜血素变异的RHD等位基因的抗-D之后的一个温暖的自身抗体。六(30%)SCD患者有变体的rhce等位基因;两种纯合变体等位基因,四个具有野生型等位基因的含有变体。结论我们展示了如何通过与血液供应商,医院和临床护理团队合作在社区医院中开发CTTP。一种掺入RBC基因分型的模型,可通知外免疫风险,并考虑转发策略以提供预防性抗原匹配血液。

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