Autoimmune hemolytic anemia

Autoimmune hemolytic anemiaAutoimmune hemolytic anemia Description, Causes and Risk Factors: Abbreviation: AIHA. Autoimmune hemolytic anemia is a group of disorders characterized by a malfunction of the immune system that produces autoantibodies, which attack red blood cells as if they were substances foreign to the body. Autoimmune hemolytic anemia is caused by autoantibody-induced hemolysis (the premature destruction of circulating red blood cells); usually idiopathic, it is also associated with infection, lymphoproliferative disorders, autoimmune diseases, and some drugs. Destruction of red blood cells by autoantibodies may occur suddenly, or it may develop gradually. In some people, the destruction may stop after a period of time; whereas in other people, it persists and becomes chronic. Autoimmune hemolytic anemia is classically divided into two groups: warm and cold disease:
  • In warm autoimmune hemolytic anemia: immunoglobulin G (IgG) attacks red blood cells (RBCs); patients are usually over age 50; typically treated with corticosteroids and therapies for underlying diseases.
  • In chronic cold agglutinin disease: cold-activated immunoglobulin M (IgM) and complement (C3d) coat RBCs and trigger hemolysis; patients usually over age 50; sometimes resolves with cold avoidance; rarely progresses to renal failure.
Hallmark findings include: anemia with elevated reticulocyte count in the absence of blood loss; a positive direct antiglobulin (Coombs) test; and spherocytes or RBC aggregates on the peripheral blood smear (PBS). There is no known genetic predisposition to AIHA, though risk factors for AIHA include a family history of autoimmune diseases, such as systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and glomerulonephritis (GN). Symptoms: Some people with autoimmune hemolytic anemia may have no symptoms, especially when the destruction of red blood cells is mild and develops gradually. Others have symptoms similar to those that occur with other types of anemia, especially when the destruction is more severe or rapid. When severe or rapid destruction of red blood cells occurs, mild jaundice may also develop. When destruction persists for a few months or longer, the spleen may enlarge, resulting in a sense of abdominal fullness and, occasionally, discomfort. When the cause of autoimmune hemolytic anemia is another disease, symptoms of the underlying disease, such as swollen and tender lymph nodes and fever, may dominate. Diagnosis:
  • The diagnosis of AIHA must meet two criteria: evidence of hemolysis (anemia plus elevated reticulocyte count in the absence of blood loss); and evidence of RBC autoantibodies/complement (usually indicated by a positive direct Coombs test). Note: the direct Coombs test is falsely negative in a small percentage of AIHA.
  • Though usually idiopathic, AIHA is commonly associated with infection, autoimmune disease, lymphoproliferative disorders, and some drugs.
  • Warm AIHA and cold agglutinin disease are most common among adults over age 50, in whom the diseases are usually chronic and relapsing; PCH, a rare disorder occurring most commonly in children, usually resolves on its own.
  • Medical intervention is usually not necessary in the many patients who present with a mild hemolytic anemia; therapy becomes necessary when anemia is significant.
Treatment: Talk with your hematologist/oncologist about the best treatment plan for you. Mild cases may not need treatment. They may resolve on their own.
  • Treating the cause of autoimmune hemolytic anemia may help treat the condition.
  • Cortisone-like drugs suppress the immune response. These drugs usually improve the more common types of autoimmune hemolytic anemia. Other immunosuppressive drugs: Other drugs that suppress the immune system may be used if corticosteroids are not effective. These include azathioprine and cyclophosphamide. Rituximab is another drug that has shown promise in treating this condition.
  • Splenectomy: The spleen removes abnormal red cells from the circulation, including those labeled with antibodies. Removing the spleen can preserve those cells and prevent anemia.
  • Transfusion: You will need transfusions if your blood gets too anemic.
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.  

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