What is hereditary spheredosis?

Hereditary spherecytosis is a genetically inherited blood disorder that results in the formation of abnormally shaped red blood cells. The individual with this disorder has spherical red blood cells instead of normal disk -shaped cells. Symptoms include anemia, fatigue, jaundice, pale skin and splenomegaly in which the spleen becomes dangerously enlarged. The greatest risk for people with hereditary spherecytosis is the low number of red blood cells, which may be life-threatening if it is sufficiently serious. These proteins are components of red blood cell membranes. Protein defects cause cell membranes to be fragile and the cells themselves have an abnormal shape. As a result, these cells are endangered by premature destruction in the spleen, because this organ is responsible for the destruction of damaged red blood cells and usually destroys cells of tklobouk is unusually shaped. In some people, the condition is asymptomatic and others have a slight anemia that deteriorates during stress disease. Some of them lead to episodesSevere hemolytic anemia in which red blood cells are destroyed faster than can be replaced. The risk that such episodes occur during stress or illness is much higher because the generation of red bone marrow blood cells slows down or stops at such times.

treatment of hereditary spherecytosis depends on the severity of the disease. Possible treatment includes monitoring red blood cell levels with transfusion in emergency situations and splenectomy in which the spleen is removed. The removal of the spleen treats most cases inherited spherocytosis, because without spleen, red blood hematopoies can not occur at abnormally high speed.

While in most cases it is therapeutic, people who have undergone this operation are exposed to an increased risk of certain types of infection. This is because in addition to its role in the destruction of red blood cells, the spleen is also an immune body that is important inRemoval of bacteria mediated by antibody from the bloodstream. The individual who has undergone splenectomy is threatened by fatal session, a condition that can develop when certain types of bacteria infect blood, urine, lungs or skin.

In people with hereditary spherecytosis, alternative surgery called partial splenectomy could be performed. Removing only parts of the spleen reduce the rate of red blood cell destruction and maintain the immunological function of the organ. In people with very serious illnesses, this option is not always appropriate, as the speed of cell destruction may be too high even after removing a partial spleen.