Contents
Definition
Sickle cell anemia is a group of hereditary Hematology which occurs especially among people from sub-Saharan Africa. heterozygous "Carriers" of the disease have some protection against Malaria infection and have few or no symptoms of the disease. homozygous (with hereditary traits from both parents) often get signs of illness as children from 5-6 months of age. The symptoms may be similar systemic lupus (SLE) in children.
Occurrence
Over 4 million people have Sigd cell anemia. About 80% come from sub-Saharan Africa. -Sygcell cell anemia.
Symptoms on sickle cell anemia
Sickle cell anemia causes many different symptoms. Most people have symptoms from childhood. Sickle cell crisis consists of episodes of severe pain, stroke, anemia (low blood percentage) and swelling of the hands and feet that may resemble rheumatic disease.
Pain in «vaso-occlusive» crisis is due to the sickle cell-shaped red blood cells not passing through narrow blood vessels. Thus arises blood clots / thrombosis and damage to bone tissue (osteonecrosis). Other serious complications are «aplastic crisis»(Low number of blood cells) and«hemolytic crisis»(Dissolution of red blood cells) where the sickle cell blood cells are destroyed. Such sickle cell crises can be triggered by infections, blood transfusions, surgeries and pregnancies in people with sickle cell anemia.
Similar conditions / Differential diagnoses
- Other hemoglobinopathies (thalassemia with more)
- Antifosfolipid syndrome (blood clots)
- Autoinflammatory Diseases (Periodic Fever Syndromes) (intermittent high fever)
- Systemic lupus (SLE) (fever, hemolysis, osteonecrosis)
- Tropical diseases
Treatment
It is important in preventing the triggering causes of sickle cell crisis. Vaccines, antibiotics for bacterial infection, copious fluid intake and strong painkillers may be appropriate. Bone marrow transplantation or gene therapy in some cases.
Prognosis
The disease (homozygous form) is severe. About half die before the age of 50.
