Outcomes on the use of hyperhemolysis prophylaxis in pediatric sickle cell disease patients with history of hyperhemolysis syndrome.

IF 2.5 3区 医学 Q2 HEMATOLOGY
Transfusion Pub Date : 2025-03-26 DOI:10.1111/trf.18227
HyoJeong Han, Lisa Hensch, In Lei, Titilope Fasipe, Jun Teruya, Shiu-Ki Rocky Hui
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Abstract

Background: Hyperhemolysis syndrome (HS) is a rare but severe transfusion-associated complication seen in patients with sickle cell disease (SCD). The management of HS includes avoidance of post-hyperhemolysis red blood cell (RBC) transfusion to avoid reoccurrence of HS (recurrent HS). However, complete avoidance of post-hyperhemolysis RBC transfusion (PHRT) is sometimes not clinically possible, and the standard of care for recurrent HS prophylaxis for patients requiring PHRT has not been established.

Case report: We present a retrospective case series of four pediatric patients with SCD and a history of HS requiring PHRT, and describe their HS prophylaxis and outcomes. All patients received HS prophylaxis before transfusion, and three patients received an additional prophylactic regimen post-transfusion. Three patients were transfused with extended phenotype-matched RBCs, while one patient received only Rh (D, C/c, E/e) and K antigens matched RBCs. Only one patient did not develop recurrent HS after PHRT. Three patients had documented hemolysis, and two patients met our criteria for recurrent HS, all requiring escalation of care.

Discussion: Even though the patients were treated in the same institution, there was variability in the choice of HS prophylaxis therapy and selection of RBCs, which can be attributed to the lack of guidance on PHRT management. We observed a lack of conclusive evidence in the effectiveness of prophylactic combination immunosuppressive therapy. Our observations suggest caution must be taken when transfusing patients with SCD and a history of HS, as there are no definitive therapies to effectively mitigate the risk of recurrent HS.

有高溶血综合征病史的儿童镰状细胞病患者预防高溶血的结果
背景:高溶血综合征(HS)是镰状细胞病(SCD)患者中一种罕见但严重的输血相关并发症。HS的管理包括避免高溶血后红细胞(RBC)输血,以避免HS(复发性HS)的再次发生。然而,完全避免高溶血后红细胞输血(PHRT)有时在临床上是不可能的,并且需要PHRT的患者复发性HS预防的护理标准尚未建立。病例报告:我们提出了4例儿童SCD患者的回顾性病例系列,并描述了他们的HS预防和结果。所有患者在输血前都接受了HS预防,三名患者在输血后接受了额外的预防方案。3例患者输注扩展表型匹配的红细胞,1例患者仅输注Rh (D、C/ C、E/ E)和K抗原匹配的红细胞。只有1例患者在PHRT后未发生复发性HS。3名患者有溶血记录,2名患者符合复发性HS的标准,所有患者都需要升级护理。讨论:尽管患者在同一机构接受治疗,但在HS预防治疗的选择和红细胞的选择上存在差异,这可归因于缺乏对PHRT管理的指导。我们观察到预防性联合免疫抑制治疗的有效性缺乏确凿的证据。我们的观察结果表明,输注有SCD和HS病史的患者时必须谨慎,因为没有明确的治疗方法可以有效地降低HS复发的风险。
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来源期刊
Transfusion
Transfusion 医学-血液学
CiteScore
4.70
自引率
20.70%
发文量
426
审稿时长
1 months
期刊介绍: TRANSFUSION is the foremost publication in the world for new information regarding transfusion medicine. Written by and for members of AABB and other health-care workers, TRANSFUSION reports on the latest technical advances, discusses opposing viewpoints regarding controversial issues, and presents key conference proceedings. In addition to blood banking and transfusion medicine topics, TRANSFUSION presents submissions concerning patient blood management, tissue transplantation and hematopoietic, cellular, and gene therapies.
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