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Sickle cell disease (SCD), also simply called sickle cell, is a group of hemoglobin-related blood disorders that are typically inherited. [2] The most common type is known as sickle cell anemia. [2] Sickle cell anemia results in an abnormality in the oxygen-carrying protein haemoglobin found in red blood cells. [2]
The underlying cause of sickle cell anemia is the synthesis of aberrant hemoglobin, which attaches to other aberrant hemoglobin molecules inside the red blood cell to undergo rigid deformation. [18] Sickle cell anemia symptoms usually appear around the age of six months. They can change over time and differ from person to person.
A vaso-occlusive crisis is a common painful complication of sickle cell anemia in adolescents and adults. [1] [2] It is a form of sickle cell crisis.Sickle cell anemia – most common in those of African, Hispanic, and Mediterranean origin – leads to sickle cell crisis when the circulation of blood vessels is obstructed by sickled red blood cells, causing ischemic injuries.
Differential diagnosis: Pyruvate kinase deficiency, hereditary spherocytosis, sickle cell anemia [2] Treatment: Avoiding triggers, medications for infection, stopping offending medication, blood transfusions [3] Frequency: 400 million [1] Deaths: 33,000 (2015) [4]
Red cell alloimmunisation is common in people with sickle cell disease who receive transfusions in Europe and North America. [4] This is because there are ethnic differences in the frequencies of blood group antigens. [4] Blood donors are usually Caucasian whereas the blood transfusion recipients usually have an African or Afro-Caribbean ancestry.
The types of anemia treated with drugs are iron-deficiency anemia, thalassemia, aplastic anemia, hemolytic anemia, sickle cell anemia, and pernicious anemia, the most important of them being deficiency and sickle cell anemia with together 60% of market share because of highest prevalence as well as higher treatment costs compared with other ...
Diamond-Blackfan Anemia is an example of a congenital bone marrow failure syndrome that primarily affects red blood cell production. In DBA, the erythroid cell lineage is more susceptible to cell death due to abnormal ribosome function. [4] This leads to a reduced population of red blood cell precursors and a resulting reticulocytopenia and anemia.
Sickle cell disease, in which a mutation in the globin gene causes the formation of sickle hemoglobin. [2] This disease is marked by the manifestation of chronic compensated hemolytic anemia, with laboratory findings not limited to unconjugated hyperbilirubinemia but also elevated serum lactate dehydrogenase and low serum haptoglobin. [2]
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