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成人镰状细胞病患者迟发性溶血性输血反应的发生率和预测评分。

Incidence and predictive score for delayed hemolytic transfusion reaction in adult patients with sickle cell disease.

机构信息

Etablissement Français du Sang, Créteil, 94000, France.

Institut Mondor de Recherche Biomédicale, lnserm U955, Equipe 2, Créteil, 94000, France.

出版信息

Am J Hematol. 2017 Dec;92(12):1340-1348. doi: 10.1002/ajh.24908. Epub 2017 Oct 31.

DOI:10.1002/ajh.24908
PMID:28924974
Abstract

Delayed hemolytic transfusion reaction (DHTR) is a life-threatening complication of transfusion in sickle cell disease (SCD). The frequency of DHTR is underestimated because its symptoms mimic those of vaso-occlusive crisis and antibodies (Abs) are often not detectable. No predictive factors for identifying patients likely to develop DHTR have yet been defined. We conducted a prospective single-center observational study over 30 months in adult sickle cell patients. We included 694 transfusion episodes (TEs) in 311 patients, divided into occasional TEs (OTEs: 360) and chronic transfusion program (CTEs: 334). During follow-up, 15 cases of DHTR were recorded, exclusively after OTEs. DHTR incidence was 4.2% per OTE (95% CI [2.6; 6.9]) and 6.8% per patient during the 30 months of the study (95% CI [4.2; 11.3]). We studied 11 additional DHTR cases, to construct a predictive score for DHTR. The DHTR mortality is high, 3 (11.5%) of the 26 DHTR patients died. The variables retained in the multivariate model were history of DHTR, number of units previously transfused and immunization status before transfusion. The resulting DHTR-predictive score had an area under the ROC curve of 0.850 [95% CI: 0.780-0.930], a negative-predictive value of 98.4% and a positive-predictive value of 50%. We report in our study population, for the first time, the incidence of DHTR, and, its occurrence exclusively in occasionally transfused patients. We also describe a simple score for predicting DHTR in patients undergoing occasional transfusion, to facilitate the management of blood transfusion in SCD patients.

摘要

迟发性溶血性输血反应(DHTR)是镰状细胞病(SCD)输血的一种危及生命的并发症。由于其症状类似于血管阻塞性危象,并且通常无法检测到抗体(Abs),因此 DHTR 的频率被低估了。尚未确定用于识别可能发生 DHTR 的患者的预测因素。我们在 30 个月的时间里进行了一项前瞻性单中心观察性研究,涉及成年 SCD 患者。我们纳入了 311 名患者的 694 次输血事件(TE),分为偶发性 TEs(OTEs:360)和慢性输血计划(CTEs:334)。在随访期间,记录了 15 例 DHTR 病例,仅在 OTE 后发生。DHTR 的发生率为每 OTE 4.2%(95%CI [2.6; 6.9]),在研究的 30 个月中,每患者 6.8%(95%CI [4.2; 11.3])。我们研究了另外 11 例 DHTR 病例,以构建 DHTR 的预测评分。DHTR 的死亡率很高,26 例 DHTR 患者中有 3 例(11.5%)死亡。多变量模型中保留的变量包括 DHTR 病史、先前输注的单位数和输血前的免疫状况。由此产生的 DHTR 预测评分的 ROC 曲线下面积为 0.850[95%CI:0.780-0.930],阴性预测值为 98.4%,阳性预测值为 50%。我们在研究人群中首次报告了 DHTR 的发生率,并且仅在偶尔输血的患者中发生。我们还描述了一种用于预测偶尔输血患者 DHTR 的简单评分,以方便 SCD 患者的输血管理。

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