Division of Hematology-Oncology, Department of Medicine, University of Pennsylvania, Philadelphia, PA.
Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, Baltimore, MD.
Blood Adv. 2024 Aug 13;8(15):3946-3960. doi: 10.1182/bloodadvances.2023011642.
Severe aplastic anemia (SAA) is a rare hematologic condition for which there is no clear management algorithm. A panel of 11 experts on adult and pediatric aplastic anemia was assembled and, using the RAND/University of California, Los Angeles modified Delphi panel method, evaluated >600 varying patient care scenarios to develop clinical recommendations for the initial and subsequent management of patients of all ages with SAA. Here, we present the panel's recommendations to rule out inherited bone marrow failure syndromes, on supportive care before and during first-line therapy, and on first-line (initial management) and second-line (subsequent management) therapy of acquired SAA, focusing on when transplant vs medical therapy is most appropriate. These recommendations represent the consensus of 11 experts informed by published literature and experience. They are intended only as general guidance for experienced clinicians who treat patients with SAA and are in no way intended to supersede individual physician and patient decision making. Current and future research should validate this consensus using clinical data. Once validated, we hope these expert panel recommendations will improve outcomes for patients with SAA.
严重再生障碍性贫血(SAA)是一种罕见的血液学疾病,目前尚无明确的治疗方案。我们组建了一个由 11 名成人和儿科再生障碍性贫血专家组成的专家组,采用 RAND/加州大学洛杉矶分校改良 Delphi 专家小组方法,对 600 多种不同的患者护理方案进行了评估,为所有年龄段 SAA 患者的初始和后续治疗制定了临床推荐意见。在这里,我们介绍专家组关于排除遗传性骨髓衰竭综合征的建议、一线治疗前和治疗期间的支持性护理、以及获得性 SAA 的一线(初始治疗)和二线(后续治疗)治疗的建议,重点讨论何时进行移植与药物治疗最恰当。这些建议是由专家组根据已发表的文献和经验达成的共识,仅供有经验的治疗 SAA 患者的临床医生参考,绝不能替代医生和患者的个体决策。当前和未来的研究应使用临床数据验证这一共识。一旦得到验证,我们希望这些专家组的推荐意见能够改善 SAA 患者的预后。