John Radcliffe Hospital, Oxford, UK.
NIHR Biomedical Research Centre, Oxford, UK.
Br J Haematol. 2020 May;189(4):635-639. doi: 10.1111/bjh.16687. Epub 2020 May 10.
With the developing COVID-19 pandemic, patients with inherited anaemias require specific advice regarding isolation and changes to usual treatment schedules. The National Haemoglobinopathy Panel (NHP) has issued guidance on the care of patients with sickle cell disease, thalassaemia, Diamond Blackfan anaemia (DBA), congenital dyserythropoietic anaemia (CDA), sideroblastic anaemia, pyruvate kinase deficiency and other red cell enzyme and membrane disorders. Cascading of accurate information for clinicians and patients is paramount to preventing adverse outcomes, such as patients who are at increased risk of fulminant bacterial infection due to their condition or its treatment erroneously self-isolating if their fever is mistakenly attributed to a viral cause, delaying potentially life-saving antibiotic therapy. Outpatient visits should be minimised for most patients, however some, such as first transcranial dopplers for children with sickle cell anaemia should not be delayed as known risk of stroke will outweigh the unknown risk from COVID-19 infection. Blood transfusion programmes should be continued, but specific changes to usual clinical pathways can be instituted to reduce risk of patient exposure to COVID-19, as well as contingency planning for possible reductions in blood available for transfusions. Bone marrow transplants for these disorders should be postponed until further notice. With the current lack of evidence on the risk and complications of COVID-19 infection in these patients, national data collection is ongoing to record outcomes and eventually to identify predictors of disease severity, particularly important if further waves of infection travel through the population.
随着 COVID-19 大流行的发展,遗传性贫血患者需要就隔离和改变常规治疗方案提供具体建议。国家血红蛋白病小组(NHP)已发布了镰状细胞病、地中海贫血、先天性红细胞生成不良性贫血(DBA)、先天性红细胞生成性卟啉病、铁幼粒细胞性贫血、丙酮酸激酶缺乏症和其他红细胞酶和膜疾病患者护理指南。将准确信息层层传递给临床医生和患者对于预防不良后果至关重要,例如,由于病情或治疗导致患者发生暴发性细菌感染的风险增加,如果其发热被误认为是病毒引起的,他们可能会错误地自我隔离,从而延误潜在的救命抗生素治疗。应尽量减少大多数患者的门诊就诊,但对于某些患者,如镰状细胞性贫血儿童的首次经颅多普勒检查不应延迟,因为已知的中风风险将超过 COVID-19 感染的未知风险。应继续进行输血计划,但可以对常规临床路径进行特定更改,以降低患者接触 COVID-19 的风险,并为可能减少可用于输血的血液做好应急计划。这些疾病的骨髓移植应推迟到另行通知。由于目前缺乏这些患者 COVID-19 感染风险和并发症的证据,正在进行全国范围内的数据收集,以记录结果,并最终确定疾病严重程度的预测因素,这在疫情再次在人群中传播时尤为重要。