Types of hemolytic anemia hemolytic anemia due to G6PD deficiency,thalassemia, idiopathic autoimmune hemolytic anemia, sickle cell anemia

a and hereditary spherocytosis. Thalassemia Thalassemia is a blood disorder passed down through families (inherited) in which the body makes an abnormal form of hemoglobin, the protein in red blood cells that carries oxygen. The disorder results in excessive destruction of red blood cells, which leads to anemia. Causes Hemoglobin is made of two proteins: Alpha globin and beta globin. Thalassemia occurs when there is a defect in a gene that helps control production of one of these proteins. There are two main types of thalassemia:

Alpha thalassemia occurs when a gene or genes related to the alpha globin protein are missing or changed (mutated). Beta thalassemia occurs when similar gene defects affect production of the beta globin protein.

There are many forms of thalassemia. Each type has many different subtypes. Both alpha and beta thalassemia include the following two forms:

Thalassemia major Thalassemia minor

You must inherit the defective gene from both parents to develop thalassemia major. Thalassemia minor occurs if you receive the defective gene from only one parent. Persons with this form of the disorder are carriers of the disease and usually do not have symptoms. Sign and Symptoms The most severe form of alpha thalassemia major causes stillbirth (death of the unborn baby during birth or the late stages of pregnancy). Children born with beta thalessemia major are normal at birth, but develop severe anemia during the first year of life. Other symptoms can include:

Bone deformities in the face

Fatigue Growth failure Shortness of breath Yellow skin (jaundice)

Persons with the minor form of alpha and beta thalassemia have small red blood cells (which are identified by looking at their red blood cells under a microscope), but no symptoms. Pathogenesis Reduced rate of synthesis of beta chains excess alpha chain precipitate in erythroblast (causing severe ineffective erythropoiesis) and in mature cells (causing haemolysis). Investigation A blood sample will be taken and sent to a laboratory for examination.

Red blood cells will appear small and abnormally shaped when looked at under a microscope. A complete blood count (CBC) reveals anemia. A test called hemoglobin electrophoresis shows the presence of an abnormal form of hemoglobin.

A test called mutational analysis can help detect alpha thalassemia that cannot be seen with hemoglobin electrophoresis. Treatment regular blood transfusions and folate supplements Bone marrow transplant

Idiopathic autoimmune hemolytic anemia Idiopathic autoimmune hemolytic anemia is a drop in the number of red blood cells due to increased destruction by the body's defense (immune) system. Causes Idiopathic autoimmune hemolytic anemia is an acquired disease that occurs when antibodies form against a person's own red blood cells. In the idiopathic form of this disease, the cause is unknown.

Idiopathic autoimmune hemolytic anemia accounts for one-half of all immune hemolytic anemias. There are other types of immune hemolytic anemias in which the cause may result from an underlying disease or medication. The disease may start quickly and be very serious. Sign and symptoms

Dark urine Fatigue Pale color (pallor) Rapid heartbeat Shortness of breath Yellow skin color (jaundice)

Investigation

Direct Coombs' test Hemoglobin in the urine Indirect Coombs' test Red blood cell count and serum hemoglobin Reticulocyte count Serum bilirubin levels Serum haptoglobin

Treatment The first therapy tried is usually a steroid medication, such as prednisone. If steroid medications do not improve the condition, removal of the spleen (splenectomy) may be considered. Therapy to suppress the immune system is usually given if the person does not respond to steroids and splenectomy. Medications such as azathioprine (Imuran), cyclophosphamide (Cytoxan), and rituximab (Rituxan) have been used. Blood transfusions are given with caution, because of the potential that blood may not be compatible and may cause further hemolysis.