Management of Transfusion‐Dependent Thalassaemia Patients in Emergency Situations
Management of Transfusion‐Dependent Thalassaemia Patients in Emergency Situations
复制标题
紧急情况下输血依赖性地中海贫血患者的管理
DOI：
10.1111/jpc.16206
复制
发表时间：
2022
影响因子：
1.7
通讯作者：
Ariyoshi Koya
中科院分区：
文献类型：
--
作者：
Aoki Yoshihiro;Smith Chris;Ariyoshi Koya
帕拉赫等人。最近针对 Covid-19 大流行以及相关封锁对印度北部输血依赖性地中海贫血 (TDT) 患者护理的影响进行了一项前瞻性观察研究。 1 他们报告称，与封锁前相比，封锁期间和封锁后的平均输血前血红蛋白水平显着降低。他们还描述了地中海贫血门诊针对Covid-19的各种问题的对策。在 Covid-19 大流行之外，例如在冲突环境中，类似的诊所可及性困难和捐献血液减少也可能发生。 2 然而，这些干扰对地中海贫血患者长期临床结果的影响尚不清楚。应进一步考虑 TDT 患者治疗中断造成的潜在危害。 9-10.5 g/dL 的输血前血红蛋白目标是否是紧急情况下地中海贫血治疗的最佳选择尚存在争议。维持一定的血红蛋白水平对于 TDT 患者至关重要，因为贫血会促进髓外造血，加剧脾肿大并增加溶血。另一方面，将基线血红蛋白维持在 10-12 g/dL 的过度输血会导致铁超负荷，并显着影响青春期延迟，因此以 9-10 g/dL 为目标的适度输血被认为更好，不会引起髓外造血。 3 然而，当考虑在资源不足的情况下的人口结果时，
Parakh et al. recently conducted a prospective observational study on the impact of the Covid-19 pandemic and the associated lockdown on the care of transfusion-dependent thalassemia (TDT) patients in North India. 1 They reported significantly lower mean pretransfusion hemoglobin levels during and post-lockdown compared to pre-lockdown. They also described the countermeasures of the thalassemia outpatient clinic to address the various issues of Covid-19. Similar difficulties in clinic accessibility and declined donor blood can occur outside of the Covid-19 pandemic, such as in conflict settings. 2 However, the impact of these disruptions on long-term clinical outcomes of thalassemia patients is not clear. Further consideration should be given to potential harm as a consequence of treatment disruption for TDT patients. Whether a pre-transfusion hemoglobin target of 9-10.5 g/dL is optimal for thalassemia treatment in emergencies is debatable. Maintenance of a certain hemoglobin level is vital in TDT patients because anemia contributes to extramedullary hematopoiesis, exacerbates splenomegaly, and increases hemolysis. On the other hand, hypertransfusion maintaining baseline hemoglobin at 10-12 g/dL contributes to iron overload and significantly affects delayed puberty, so moderate transfusion aiming for 9-10 g/dL has been considered better without causing extramedullary hematopoiesis. 3 However, when considering population outcomes in situations of insufficient
影响因子：
0.5
作者：
Wiwanitkit, Somsri;Wiwanitkit, Viroj
通讯作者：
Wiwanitkit, Viroj