Molecular Haematology, Faculty of Life Sciences & Medicine, King's College London, London, UK.
Department of Haematological Medicine, King's College Hospital NHS Foundation Trust, London, UK.
Br J Haematol. 2015 Jun;169(5):746-53. doi: 10.1111/bjh.13339. Epub 2015 Mar 5.
Delayed haemolytic transfusion reactions (DHTR) are potentially life-threatening complications in patients with sickle cell disease (SCD). Between 1 August 2008 and 31 December 2013, 220 of 637 adult patients in our centre had at least one red blood cell (RBC) transfusion in 2158 separate transfusion episodes. Twenty-three DHTR events occurred in 17 patients (13 female) including 15 HbSS, one HbSC and one HbSβ(0) thalassaemia, equating to a DHTR rate of 7·7% of patients transfused. Mean interval from RBC transfusion to DHTR event was 10·1 ± 5·4 d, and typical presenting features were fever, pain and haemoglobinuria. Twenty of the 23 (87·0%) DHTR episodes occurred following transfusion in the acute setting. Notably, 11/23 (47·8%) of DHTRs were not diagnosed at the time of the event, most were misdiagnosed as a vaso-occlusive crisis. 16/23 DHTRs had 'relative reticulocytopenia', which was more common in older patients. Seven of 23 episodes resulted in alloantibody formation, and three caused autoantibody formation. DHTRs are a severe but uncommon complication of RBC transfusion in SCD and remain poorly recognized, possibly because they mimic an acute painful crisis. Most of the DHTRs are triggered by RBC transfusion in the acute setting when patients are in an inflammatory state.
迟发性溶血性输血反应（DHTR）是镰状细胞病（SCD）患者潜在的危及生命的并发症。在 2008 年 8 月 1 日至 2013 年 12 月 31 日期间，我们中心的 637 名成年患者中有 220 名患者在 2158 次单独的输血事件中至少接受了一次红细胞（RBC）输血。在 17 名患者（13 名女性）中发生了 23 例 DHTR 事件，包括 15 例 HbSS、1 例 HbSC 和 1 例 HbSβ（0）地中海贫血，DHTR 发生率为接受输血的患者的 7.7%。从 RBC 输血到 DHTR 事件的平均间隔为 10.1±5.4d，典型的表现特征是发热、疼痛和血红蛋白尿。23 例 DHTR 中有 20 例（87.0%）发生在急性输血后。值得注意的是，11/23（47.8%）例 DHTR 在事件发生时未得到诊断，大多数被误诊为血管阻塞危象。23 例 DHTR 中有 16 例出现“相对网织红细胞减少症”，在老年患者中更为常见。7 例 DHTR 导致同种抗体形成，3 例导致自身抗体形成。DHTR 是 SCD 中 RBC 输血的严重但不常见的并发症，仍然认识不足，可能是因为它们类似于急性疼痛危机。大多数 DHTR 是在患者处于炎症状态的急性情况下由 RBC 输血引发的。
