La Revue de médecine interne
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Worsening of anemia is very common in sickle cell disease. It is important to investigate specific complications related to sickle cell disease but also other causes of anemia in general. Transfusions or exchange transfusions are major therapeutic options and are frequently used for acute complications of sickle cell disease but also for primary and secondary prevention of some of the chronic complications. ⋯ A strong collaboration between the patient's expert center, the Blood center and the patient's hospitalization unit is required to make decisions. © 2023 Société nationale française de médecine interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.
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Sickle Cell Anemia is a disease with a strong vascular tropism. Beyond anemia, the pathophysiological mechanisms responsible for hemolysis, directly affect both acute and chronic vascular damages, thus resulting in a systemic disease. Understanding the different types of hemolysis underline the need for novel specific biomarkers. ⋯ It seems likely that the rapidly evolving knowledge in this field will also benefit other diseases. © 2023 Société nationale française de médecine interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.
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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. ⋯ 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.