Presse Med
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Pregnancy is a particularly risky period in the life of patients with sickle cell disease (SCD). Physiological changes during pregnancy increase the risk of vaso-occlusive crises (VOC), acute chest syndrome, venous thromboembolic events, and infections. This concerns haemoglobin (Hb) S/C and S/β+-thalassaemia patients as much than S/S or S/β0-thalassaemia patients. ⋯ Although more frequent, due to obstetrical and maternal complications, caesarean section is not systematic, in the absence of maternal contraindications. It is advisable not to exceed the term of 39 weeks of amenorrhoea. Post-partum follow-up is recommended, particularly because of the risk of thromboembolism.
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Neurovascular complications represent one of the most detrimental manifestations of Sickle Cell Disease (SCD), affecting many patients since infancy. They include overt stroke, silent cerebral infarcts and neurocognitive disorders. In fact, neurodevelopment can be impaired in children resulting in cognitive dysfunction in adults with SCD. ⋯ Chronic transfusion regimen, hematopoietic stem cell transplantation and neurocognitive rehabilitation find indications in the context of primary and secondary prevention of neurovascular complications of SCD. However, international guidelines are often difficult to bring into the real world due to the lack of appropriate instruments and trained personnel. Many challenges have still to be faced to guarantee the best possible neurocognitive function to each child affected by SCD.
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Sickle cell disease (SCD) is an hereditary disorder characterized by the production of an abnormal hemoglobin called hemoglobin S (HbS). HbS may polymerize in deoxygenated conditions, which leads to red blood cell (RBC) sickling. Sickled RBCs are more rigid and fragile, and prone to lysis. ⋯ Furthermore, chronic hemolysis is responsible for increased inflammation and oxidative stress, which affect the vascular system and may promote the adhesion of circulating cells to endothelial cells. Extracellular vesicles and especially RBC microparticles (massively released in the context of SCD) are also at the origin of vascular damages and increased white blood cells adhesion to the endothelium, which may trigger vaso-occlusive crisis and other vascular-related complications. This review highlights the fact that SCD should not only be considered as a hematological disorder but also as a vascular disease.
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Sickle Cell Disease (SCD) is a hereditary blood disorder characterized by the presence of abnormal hemoglobin, leading to the formation of sickle-shaped red blood cells, causing vaso-occlusion. Inflammation is a key component of the pathophysiology of SCD, contributing to the vascular complications and tissue damage. ⋯ Additionally, we will discuss the proposed mechanisms by which the complement system may contribute to tissue injury in this pathology. Finally, we will provide an overview of the available evidence concerning the effectiveness of therapeutic interventions aimed at blocking the complement system in the context of SCD and discuss the perspective of complement inhibition.