Department of Pediatrics, Division of Pediatric Hematology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Pediatric Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA.
Transfusion. 2024 Aug;64(8):1509-1519. doi: 10.1111/trf.17924. Epub 2024 Jul 14.
The data to support chronic automated red cell exchange (RCE) in sickle cell disease (SCD) outside of stroke prevention, is limited, especially in adults.
A retrospective analysis was conducted of patients with SCD who were referred for chronic RCE at our institution over a 10-year period. Data that were evaluated included patient demographics, referral indications, and procedural details (e.g., vascular access, adverse events, etc.). In a subanalysis, the number of annual acute care encounters during 3 years of chronic RCE was compared with that in the year preceding the first RCE.
A total of 164 patients were referred for chronic RCE: median age was 28 years (interquartile range [IQR] = 22-36) at referral and 60% were female. Seventy (42.6%) were naïve to chronic transfusion (simple or RCE) prior to referral. The leading indications for referral were refractory pain (73/164, 44.5%) and iron overload (57/164, 34.7%). A total of 5090 procedures occurred during the study period (median = 19, IQR = 5-45). Of the 138 patients who had central vascular access, 8 (6%) and 16 (12%) had ≥1 central-line-related thrombosis and/or infection, respectively. Of those who were not RBC alloimmunized at initiation of RCE, 12/105 (11.4%) developed new antibodies during chronic RCE. In those 30 patients who were adherent to therapy for 3 years, there was no significant difference in acute care encounters following initiation of RCE.
Prospective clinical trials are needed to determine which patients are most likely to benefit from chronic RCE and refine selection accordingly.
在预防中风之外，慢性自动红细胞交换（RCE）在镰状细胞病（SCD）中的应用数据有限，尤其是在成人中。
对在我们机构进行慢性 RCE 转介的 SCD 患者进行了回顾性分析，时间跨度为 10 年。评估的数据包括患者人口统计学、转介指征和程序细节（例如血管通路、不良事件等）。在亚分析中，比较了慢性 RCE 前 3 年的每年急性护理就诊次数与 RCE 前 1 年的就诊次数。
共有 164 名患者被转介进行慢性 RCE：转介时的中位年龄为 28 岁（四分位距[IQR]22-36），60%为女性。70 名（42.6%）患者在转介前从未接受过慢性输血（单纯或 RCE）。转诊的主要指征是难治性疼痛（73/164，44.5%）和铁过载（57/164，34.7%）。研究期间共进行了 5090 次手术（中位数=19，IQR=5-45）。在有中心血管通路的 138 名患者中，分别有 8 名（6%）和 16 名（12%）患者发生了≥1 次中心静脉导管相关血栓形成和/或感染。在开始 RCE 时未发生 RBC 同种免疫的 105 名患者中，有 12 名（11.4%）在慢性 RCE 期间发生了新抗体。在 30 名坚持治疗 3 年的患者中，RCE 开始后急性护理就诊次数没有显著差异。
需要进行前瞻性临床试验来确定哪些患者最有可能从慢性 RCE 中受益，并相应地进行选择。
