Fort Romain
Department of Internal Medicine, CHU Edouard Herriot, Lyon, France; Laboratoire LIBM EA7424, Equipe "Biologie Vasculaire et du Globule Rouge", Université Claude Bernard, Lyon, France; Laboratoire d'Excellence du Globule Rouge (LABEX GR-Ex), PRES Sorbonne, Paris, France.
Transfus Apher Sci. 2019 Apr;58(2):128-131. doi: 10.1016/j.transci.2019.03.004. Epub 2019 Mar 13.
Sickle cell disease (SCD) is a genetic disorder characterised by a single mutation of the beta globin gene, causing the production of an abnormal haemoglobin called sickle haemoglobin (HbS). In its deoxygenated form, HbS polymerises, causing major rheological disorders, which presents clinically as periodic vaso-occlusive crises, chronic haemolysis and chronic vascular dysfunction. Patients often resort to a background treatment, and transfusion remains the cornerstone in the management of the disease, significantly reducing morbidity and mortality. The aim of red blood cell exchange (RBCX) is to improve tissue oxygenation by increasing haemoglobin levels while lowering HbS levels. RBCX can be performed by manual or automated exchange, and each technique has its own set of advantages and disadvantages. This article will outline the transfusion indications for the main complications of SCD, as well as the most appropriate strategy to use.
镰状细胞病（SCD）是一种遗传性疾病，其特征是β珠蛋白基因发生单一突变，导致产生一种名为镰状血红蛋白（HbS）的异常血红蛋白。在其脱氧形式下，HbS会聚合，引发严重的流变学紊乱，临床上表现为周期性血管闭塞性危机、慢性溶血和慢性血管功能障碍。患者通常需要进行基础治疗，输血仍然是该疾病管理的基石，可显著降低发病率和死亡率。红细胞置换（RBCX）的目的是通过提高血红蛋白水平同时降低HbS水平来改善组织氧合。RBCX可通过手动或自动置换进行，每种技术都有其自身的优缺点。本文将概述SCD主要并发症的输血指征以及最合适的使用策略。
