Sickle cell disease

General or Other | Hematology | Sickle cell disease (Disease)


Description

Sickle cell anemia (cell disease) or sickle cell anemia is an inherited disorder involving the production of hemoglobin S (Hgb S or Hb S), an abnormal type of hemoglobin. Hemoglobin is a protein that contains iron, located inside red blood cells. It carries oxygen from the lungs to all parts of the body and provides oxygen to all body cells and tissues.

Normally, hemoglobin F is produced during gestation of the fetus. After the baby is born, Hb F is replaced gradually by hemoglobin A (Hb A). Other types of hemoglobin except Hb F and Hb A derived from genetic mutations. Several variants have been identified, but few are common. These include changes associated with Hb S, Hb C and beta thalassemia. If a person has a normal copy of the hemoglobin gene and one copy of Hb S gene, there is a production of 20-40% of Hb S, but will produce enough hemoglobin A. Therefore, there will be problems involved health.

Symptoms and complications of sickle cell disease may be experienced by people who have a copy of the sickle cell gene and one altered gene copy of another abnormal hemoglobin variants (double heterozygous), such as hemoglobin C or thalassemia. For all those who have two copies of the gene HbS (SS) and those with one copy of a variant (SC, beta talesemie, SD, SOArab) it is present in the form of sickle cell or events specified siclemiei siclemie. Other symptoms include: Fatigue and anemia; Attacks of pain; Dactylitis (swelling and inflammation of the hands or feet); Arthritis; Bacterial; Splenic sequestration; Congestion of liver; Damage to the lungs and heart; Leg ulcers; Aseptic necrosis and bone infarction; Diseases of the eye; Other symptoms.

Causes and Risk factors

Sickle cell anemia is caused by an abnormal type of hemoglobin called hemoglobin S. Hemoglobin is a protein inside red blood cells that carries oxygen. Hemoglobin S changes the shape of red blood cells. The red blood cells become shaped like crescents or sickles. The fragile, sickle-shaped cells deliver less oxygen to the bodys tissues. They can also get stuck more easily in small blood vessels, as well as break into pieces that can interrupt healthy blood flow. These problems decrease the amount of oxygen flowing to body tissues even more.

Sickle cell anemia is inherited from both parents. If you inherit the sickle cell gene from only one parent, you will have sickle cell trait. People with sickle cell trait do not have the symptoms of sickle cell anemia.

Diagnosis and Treatment

Tests will be performed to diagnose and monitor the disease. The goal of therapy is to control symptoms, and to limit the frequency of crises. Hydroxyurea (Hydrea) is used to reduce the acute episodes of pain but is not effective in all patients. An acute episode is treated with the following: intravenous fluids, supplemental oxygen, pain medications, antibiotics (if infection is found), and/or blood tra...