患有镰状细胞病并有儿童中风史的长期输血成人的纵向疗效。

IF 2.5 3区医学 Q2 HEMATOLOGY Transfusion Pub Date : 2024-11-05 DOI:10.1111/trf.18041

Jennifer M Jones, Julia Wool, Elizabeth P Crowe, Evan M Bloch, Lydia H Pecker, Sophie Lanzkron

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引用次数: 0

摘要

背景：许多中风的镰状细胞病（SCD）患儿会无限期地接受慢性输血治疗（CTT）；然而，他们成年后的神经系统预后尚未见报道。了解这些结果对儿童期治疗决策至关重要：在这项回顾性研究中，我们描述了在一个中心接受治疗的一组患有 SCD 且有儿童期中风史的成人，并使用 chi2 和 Mann-Whitney U 检验比较了他们与无儿童期中风的匹配受试者的预后：结果：在42名有儿童中风史的患者中，所有患者都接受了CTT治疗，以预防继发性中风。5人（11%）再次发生中风。儿童中风患者和非儿童中风患者的中风发生率相似（0.7 对 1.1/100人年，p = .63）。尽管接受了综合 SCD 项目的治疗，但两组患者都表现出铁超载（铁蛋白中位数为 2227 vs. 1409 ng/dL，p = .10）和同种免疫（45% vs. 45%，p = 1.0）：讨论：对于儿童期中风的成人 SCD 患者，我们的研究结果表明 CTT 可使中风风险恢复到与年龄匹配的中风幼稚 SCD 患者的水平。

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Longitudinal outcomes of chronically transfused adults with sickle cell disease and a history of childhood stroke.

Background: Many children with sickle cell disease (SCD) who suffer a stroke receive chronic transfusion therapy (CTT) indefinitely; however, their adulthood neurologic outcomes have not been reported. Understanding these outcomes is critical to inform decisions regarding curative therapy in childhood.

Study design and methods: In this retrospective study, we described a cohort of adults with SCD and a history of childhood stroke who received care at a single center and compared their outcomes with matched subjects without childhood stroke using chi² and Mann-Whitney U tests.

Results: Of 42 subjects with childhood stroke, all received CTT for secondary stroke prophylaxis. Five (11%) developed recurrent stroke. The rate of stroke was similar in subjects with and without childhood stroke (0.7 vs. 1.1 per 100 person·years, p = .63). Both cohorts exhibited evidence of iron overload (median ferritin 2227 vs. 1409 ng/dL, p = .10) and alloimmunization (45% vs. 45%, p = 1.0), despite receiving care in a comprehensive SCD program.

Discussion: For adults with SCD who had a childhood stroke, our results suggest CTT returns the risk of stroke to that of age-matched stroke naïve patients with SCD.

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来源期刊

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.