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 inheritance from both parents) often get sickness signs as children from 5-6 months of age.
Occurrence
Over 4 million people have Sickle Cell Anemia, the vast majority in sub-Saharan Africa.
Symptoms
Sickle cell anemia causes many different symptoms. Sickle cell crisis consists of episodes of severe pain, stroke, anemia (low blood percentage) and swelling of the hands and feet.
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 og osteonecrosis. Aplastic crisis (low number of blood cells) and hemolytic crisis (hemolysis of red blood cells) is caused by the destruction of the sickle cell blood cells. Such sickle cell crises can be triggered by infections, blood transfers, surgeries and pregnancies.
Similar conditions / Differential diagnoses
- Other hemoglobinopathies (thalassemia with more)
- Tropical diseases
- Autoinflammatory Diseases (Periodic Fever Syndromes)
- Systemic lupus (SLE)
Treatment
It is important to prevent triggering causes of sickle cell crisis. Vaccines, antibiotics for bacterial infection, ample fluid intake and strong painkillers may be appropriate. Bone marrow transplant or gene therapy in some cases.
Prognosis
The disease (homozygous form) is severe. About half die before age 50
