The process of the destruction of red blood cells in the body is called hemolysis. Hemolytic anemia is a condition that occurs when the cycle of red blood cell destruction and replacement gets disrupted. Accelerated destruction of red blood cells before the bone marrow can produce enough new ones to replace them leads to the presence of fewer red blood cells in the body to transport oxygen to various tissues in the body. This puts a strain on the heart to work harder so that oxygen-rich blood reaches all parts of the body. There are several factors, medical conditions, or diseases that may be acquired or inherited that lead to the premature destruction of red blood cells. While the inherited form of hemolytic anemia may remain a lifelong condition, acquired hemolytic anemia often responds to treatment when the cause of the condition is addressed. Weakness, fatigue, and lack of energy are usually associated with hemolytic anemia since oxygen supply to tissues all over the body is affected. Those with severe forms of inherited hemolytic anemia, which is also sometimes referred to as intrinsic anemia, usually inherit one defective gene from each parent. Sickle cell anemia and thalassemia patients are the most common among those with inherited hemolytic anemia.
Acquired hemolytic anemia, also classified as extrinsic, may be the result of infections, certain types of medications, auto-immune disorders, tumors, or leukemia. Incompatibility of donor blood with the recipient's blood is also one of the causes of accelerated destruction of red blood cells.
Patients with hemolytic anemia may experience severe or mild symptoms, depending on the individual level of hemolysis and how successful the bone marrow is in replacing the destroyed red blood cells. Among congenital hemolytic anemia the most common is the deficiency of the enzyme glucose-6-phosphate-dehydrogenase, popularly known as G6PD. Cells with reduced amounts of this G6PD are likely to breakdown when the individual is exposed to the stress of an infection.
A physical examination by a medical practitioner is the first step to check for signs of hemolytic anemia. The doctor examines the skin and the whites of the eyes for yellow coloring to rule out jaundice. The physical examination will also reveal if the spleen is enlarged or if there is heart murmur or labored breathing. Here are some of the hemolytic anemia tests that the doctor may order:
For males with hemolytic anemia, mental tests may be ordered in case of phosphoglycerate kinase deficiency, which results in varying degrees of mental retardation accompanied by an enlarged spleen and hemolytic anemia.
Acquired hemolytic anemia causes could be serious infections such as hepatitis, typhoid, E-Coli, or even streptococcus. This type of autoimmune hemolytic anemia occurs when normal red blood cells get destroyed by the infection or by antibodies generated by the body's own immune system. In the case of immune disorders like rheumatoid arthritis, ulcerative colitis, or lupus, there may be an improvement in the condition from time to time when the disease causing the anemic condition is under control.
Certain toxic chemicals in drugs like penicillin, sulfa drugs, acetaminophen, and anti-malarial medication may be the reason for the destruction of red blood cells before the end of their normal life cycle, and the red blood cell count may improve once the medication is discontinued. Intrinsic hemolytic anemia causes refer to abnormalities in proteins that build red blood cells. It is a genetic abnormality that is often inherited when a child gets a defective gene from each parent, as in the case of thalassemia patients.
Other hereditary causes for anemia could be hereditary elliptocytosis, hereditary spherocytosis, hereditary ovalocytosis, which are all characterized by the abnormal shape of the red blood cells. These abnormally shaped red blood cells are more fragile and break down sooner. Hemolytic anemia could also be caused by malarial parasites, which breed inside red blood cells and then destroy the infected red blood cells as they come out and spread to other cells. Hence, the need to get blood tests done if malaria is suspected. Exposure to toxic chemicals, venom, radiation, or a burn accident can also lead to hemolytic anemia in some. The incompatibility of the Rh factor in the blood of a mother and a fetus can result in the destruction of red blood cells in the fetus since the mother's blood develops antibodies to destroy them.
Jaundice is one of the visible hemolytic anemia symptoms that prompts an immediate blood test, so as to confirm the diagnosis. When red blood cells are destroyed, the hemoglobin in them is released into the blood, and it is eventually recycled to make fresh red blood cells. Bilirubin is an unusable by-product of this process,and it normally gets eliminated by the action of the liver and the kidneys through urine. When too many red blood cells are destroyed and the liver and kidneys cannot eliminate bilirubin at the same rate, it accumulates under the skin and in the whites of the eyes, turning them yellow. Among hemolytic anemia signs and symptoms, pale, yellow skin caused by the accumulation of bilirubin in the body is the easiest to recognize. Another symptom is upper abdominal pain as a result of gall stones. Theses gall stones could form due to excess cholesterol and bilirubin in the body. The spleen is the organ where red blood cells are screened and destroyed at the end of their life cycle, and an enlarged spleen is a symptom that it is working over time. Diseases that affect the spleen or cause damage to the spleen during an injury can also result in the untimely destruction of red blood cells. Lethargy, nausea, and dizziness are often symptoms when hemolytic anemia is caused by G6PD deficiency. Swollen extremities, puffy eyelids, and difficulty in breathing are some hemolytic anemia symptoms children with vitamin E deficiency have been observed to experience. It is important to take steps to rectify such deficiencies as early as possible since neglect of these symptoms can result in long term irreversible damage like bone deformation. When fever, headaches, nausea, and chills are followed by pale skin and fatigue it is quite likely that the person is suffering from malaria and the anemia has been caused by this infection.
Patients with a mild form of acquired hemolytic anemia respond well to treatment. Hemolytic anemia treatments for those with deficiency of vitamin E or the enzyme G6PD are often successful in relieving the patients of their symptoms. Cell membranes are said to become weaker and rupture easily when the body is deficient in vitamin E, and supplements of the vitamin can help to improve the condition. Those who have congenital G6PD deficiency can avoid chemicals that trigger the anemia. Specialists like hematologists may be approached for an anemia consultation to chart out the course of treatment over a period of time, particularly for those with sickle cell anemia or thalassemia.
Some experts recommend folic acid administration as part of the treatment for anemia, but it is effective largely in cases of deficiencies during pregnancy. Transfusion therapy may be suggested for patients with severe anemia or where the condition may prove fatal without the transfusion. Type matching and cross matching of blood and donor is vital, but may be difficult in the case of auto immune hemolytic anemia or AIHA. In patients with intravascular hemolysis, iron therapy may be required to replenish depleted iron stores. A surgical procedure to remove the spleen, called splenectomy surgery, may be advised in those cases where the spleen is enlarged or diseased. In fact, in cases of hereditary spherocytosis, hereditary elliptocytosis, splenectomy may help reduce the high rate of destruction of red blood cells. Thalassemia and sickle cell anemia patients may have to undergo this surgery when other treatments like blood and bone marrow stem cell transplants fail.