Sickle cell disease | |
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Other names | Sickle cell disorder; drepanocytosis (dated) |
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Figure (A) shows normal red blood cells flowing freely through a blood vessel. The inset shows a cross-section of a normal red blood cell with normal haemoglobin. Figure (B) shows abnormal, sickled red blood cells sticking at the branching point in a blood vessel. The inset image shows a cross-section of a sickle cell with long polymerized sickle haemoglobin (HbS) strands stretching and distorting the cell shape to look like a crescent moon. | |
Specialty | Hematology, medical genetics |
Symptoms | Attacks of pain, anemia, swelling in the hands and feet, bacterial infections, stroke[1] |
Complications | Chronic pain, stroke, aseptic bone necrosis, gallstones, leg ulcers, priapism, pulmonary hypertension, vision problems, kidney problems[2] |
Usual onset | 5–6 months of age[1] |
Causes | Genetic, Homozygous mutation in the hemoglobin S gene.[3] |
Risk factors | West African, Equatorial African,[4] Central Indian,[5] Baharna or Eastern Saudi Arabian[6] ancestry[7] |
Diagnostic method | Blood test[8] |
Treatment | Vaccination, antibiotics, high fluid intake, folic acid supplementation, pain medication, blood transfusions[9][10] |
Prognosis | Life expectancy 40–60 years (developed world)[2] |
Frequency | 7.7 million (2021)[11] |
Deaths | 34,000 p.a. (a contributory factor to a further 376,000 p.a.)[11] |
Sickle cell disease | |
---|---|
Other names | Sickle cell disorder; drepanocytosis (dated) |
![]() |
|
Figure (A) shows normal red blood cells flowing freely through a blood vessel. The inset shows a cross-section of a normal red blood cell with normal haemoglobin. Figure (B) shows abnormal, sickled red blood cells sticking at the branching point in a blood vessel. The inset image shows a cross-section of a sickle cell with long polymerized sickle haemoglobin (HbS) strands stretching and distorting the cell shape to look like a crescent moon. | |
Specialty | Hematology, medical genetics |
Symptoms | Attacks of pain, anemia, swelling in the hands and feet, bacterial infections, stroke[1] |
Complications | Chronic pain, stroke, aseptic bone necrosis, gallstones, leg ulcers, priapism, pulmonary hypertension, vision problems, kidney problems[2] |
Usual onset | 5–6 months of age[1] |
Causes | Genetic, Homozygous mutation in the hemoglobin S gene.[3] |
Risk factors | West African, Equatorial African,[4] Central Indian,[5] Baharna or Eastern Saudi Arabian[6] ancestry[7] |
Diagnostic method | Blood test[8] |
Treatment | Vaccination, antibiotics, high fluid intake, folic acid supplementation, pain medication, blood transfusions[9][10] |
Prognosis | Life expectancy 40–60 years (developed world)[2] |
Frequency | 7.7 million (2021)[11] |
Deaths | 34,000 p.a. (a contributory factor to a further 376,000 p.a.)[11] |