Sickle cell disease is syndromic, associating a hemolytic anemia, a vaso-obstructive vascular disease, and an infectious risk linked to the precocity of the splenic function loss. The willingly hyperacute and potentially fatal character of the two last elements of the pathophysiologic syndrome, has, quite rightly, focused the therapeutic researches on them. Great success in those two domains have allowed a very important gain in life expectancy. However, chronic progressive organ dysfunction began to appear in older than 25 years-old patients. It concerns mainly renal, hepatic, cardiac functions and pulmonary arterial pressure and may lead to organ failure and premature death. Since some 25 years, the clinical research demonstrated an association between such complications and intravascular hemolytic rate, and it turned to a causative relationship. This present paper try to summarize the actual knowledge on the structural and genetic aspects of sickle cell anemia hemolysis. © 2023 Société nationale française de médecine interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

Sickle cell anemia is a genetic disorder that affects hemoglobin leading to the production of an abnormal hemoglobin, called HbS. HbS has the property to polymerize under deoxygenated conditions, causing a mechanical distortion of red blood cells; a phenomenon called sickling. These sickle red blood cells are more fragile and rigid, leading to chronic hemolytic anemia and painful vaso-occlusive crises, as well as chronic vascular complications that can affect many organs. The abnormal functional properties of these sickle red blood cells are responsible for a wide range of clinical expression of the disease. HbS polymerization can be influenced by many factors, such as the hydration state of the red blood cells or the affinity of hemoglobin for oxygen. Moreover, the rheological characteristics of red blood cells, including their deformability and aggregation properties, are associated with specific clinical phenotypes. The pro-inflammatory and pro-oxidant state, as well as the repeated polymerization of HbS, accelerate the senescence of sickle red blood cells, promoting the release of microparticles and contributing to vascular dysfunction. Patients\' red blood cells also have molecular characteristics that promote their adhesion to the endothelium and other circulating cells, contributing to the onset of vascular complications. Massive intravascular hemolysis, due to increased erythrocyte fragility, is also responsible for chronic vascular complications. These different alterations are privileged therapeutic targets, leading to the emergence of new specific treatments. © 2023 Société nationale française de médecine interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

Platelet transfusion in patients, particularly in onco-haematology, is frequent and can become chronic in some cases. Post-transfusion alloimmunization is often seen, in practice. The risk of this is significantly improved in multitransfused patients. Several classes of antigens binding on platelets (HLA and HPA) are involved and also red blood cell antigens (residual red blood cells in platelet concentrates). Platelet alloimmunization causes a poor transfusion response, refractoriness and, more rarely, post-transfusion purpura. In an alloimmunized recipient, the efficiency of platelet transfusion is based on the selection of compatible products. Significant technical progress means that several methods are currently available to ensure a good post-transfusion platelet count and a satisfactory clinical outcome for the patient.

It is well recognized that anaemia, a frequent complication of critical illness, is associated with poor outcomes, perhaps particularly in patients with ischaemic heart disease. But studies have also reported increased morbidity and mortality in patients who receive blood transfusions. So which carries the biggest risk, when should we transfuse and when should we hold off? Should we have fixed transfusion triggers and if so in all patients, or different triggers for different groups of patients? Indeed, these are more complex decisions than initially apparent. ICU patients are very heterogeneous and will react differently to the same intervention. As such, decisions to transfuse or not must be individualized, taking into account specific patient factors, such as age and comorbidities, physiologic variables, as well as the haemoglobin value. This approach will ensure that anaemia is treated when necessary while avoiding unnecessary exposure to red blood cells.