American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support
American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support
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DOI：
10.1182/bloodadvances.2019001143
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发表时间：
2020-01-28
期刊：
影响因子：
7.5
通讯作者：
Akl, Elie A.
中科院分区：
文献类型：
--
作者：
Chou, Stella T.;Alsawas, Mouaz;Akl, Elie A.
背景：红细胞输注仍然是镰状细胞病（SCD）患者的主要治疗方法，但也带来了重大的临床挑战。指导输血的具体适应症和管理，以及同种免疫、迟发性溶血性输血反应（DHTRs）和铁超负荷的筛查、预防和管理可能会改善结局。目的：我们的目标是制定基于证据的指南来支持患者，临床医生，和其他医疗保健专业人员对SCD的输血支持和输血相关并发症的管理的决策。美国血液学会成立了一个多学科小组，该小组是平衡的，以尽量减少利益冲突的偏见，其中包括一名患者代表。专家组优先考虑临床问题和结果。马约诊所循证实践研究计划支持指南的制定过程。采用建议评估、制定和评价分级（GRADE）方法形成建议，并接受公众评论。结果：专家组制定了10项建议，重点是红细胞抗原分型和匹配、适应症和给药方式（简单与红细胞交换），以及同种免疫、DHTR和铁超载的筛查、预防和管理。大多数专家组的建议是有条件的，因为缺乏直接的、高确定性的证据来证明感兴趣的结果。确定了研究优先事项，包括前瞻性研究，以了解血清学与基因型红细胞匹配的作用、特定同种抗原产生HTR的机制以指导治疗、女性怀孕期间定期输血的作用和时机，以及最佳治疗方法SCD中输血铁超载。
Background: Red cell transfusions remain a mainstay of therapy for patients with sickle cell disease (SCD), but pose significant clinical challenges. Guidance for specific indications and administration of transfusion, as well as screening, prevention, and management of alloimmunization, delayed hemolytic transfusion reactions (DHTRs), and iron overload may improve outcomes.Objective: Our objective was to develop evidence -based guidelines to support patients, clinicians, and other healthcare professionals in their decisions about transfusion support for SCD and the management of transfusion -related complications.Methods: The American Society of Hematology formed a multidisciplinary panel that was balanced to minimize bias from conflicts of interest and that included a patient representative. The panel prioritized clinical questions and outcomes. The Mayo Clinic Evidence -Based Practice Research Program supported the guideline development process. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach was used to form recommendations, which were subject to public comment.Results: The panel developed 10 recommendations focused on red cell antigen typing and matching, indications, and mode of administration (simple vs red cell exchange), as well as screening, prevention, and management of alloimmunization, DHTRs, and iron overload.Conclusions: The majority of panel recommendations were conditional due to the paucity of direct, high certainty evidence for outcomes of interest. Research priorities were identified, including prospective studies to understand the role of serologic vs genotypic red cell matching, the mechanism of HTRs resulting from specific alloantigens to inform therapy, the role and timing of regular transfusions during pregnancy for women, and the optimal treatment of transfusional iron overload in SCD.