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Human leukocyte antigens

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Also listed as: WAS
Related terms
Background
Author information
Bibliography
Hla-b27
Hla-dr4
Histocompatibility antigen test

Related Terms
  • Antibodies, auto recessive, B-cells, bone marrow, bone marrow transplant, CBC, genetic disorder, immune system, immunodeficiency, inherited disorder, inherited immunodeficiency, leukocytes, leukemia, lymphoma, lymphocytes, malignancy, platelets, pneumonia, red blood cells, T-cells, thrombocytes, thrombocytopenia, tumor, WASP, white blood cells, Wiskott Aldrich syndrome, Wiskott-Aldrich syndrome protein, X-linked.

Background
  • Wiskott-Aldrich syndrome (WAS) is an inherited, immunodeficiency disorder that occurs almost exclusively in males. The recessive genetic disorder is caused by a mutation in the WAS (Wiskott-Aldrich syndrome) gene, which is an X-linked trait. The gene mutation leads to abnormalities in B- and T-lymphocytes (white blood cells), as well as blood platelet cells. In a healthy individual, the T-cells provide protection against viral and fungal infection, the B cells produce antibodies, and platelets are responsible for blood clotting to prevent blood loss after a blood vessel injury.
  • Individuals diagnosed with WAS suffer from recurrent infections, eczema and thrombocytopenia (low levels of platelets).
  • Before 1935, patients only lived an average of eight months. Today, patients usually live an average of eight years, according to a recent case study. The cause of death is usually attributed to extensive blood loss. However, cancer (especially leukemia) is common and often fatal among WAS patients.
  • The only possible cure for WAS is a bone marrow transplant. However, if a patient's family member is not a possible match for a bone marrow donation, patients may have to wait years for a potential donor. Other aggressive treatments may also increase a patient's life expectancy. For instance, one study found that patients who underwent splenectomy (removal of the spleen) lived to be more than 25 years old. The spleen may harbor too many platelets, and cause a decrease in the number of platelets in circulation. Antibiotics, antivirals, antifungals, chemotherapeutic agents, immunoglobulins and corticosteroids have also been used to relieve symptoms and treat infections and cancer associated with WAS.
  • Researchers estimate that about four people per one million live male births develop the disease in the United States.
  • The syndrome is named after Dr. Robert Anderson Aldrich, an American pediatrician who described the disease in a family of Dutch-Americans in 1954, and Dr Alfred Wiskott, a German pediatrician who discovered the syndrome in 1937. Wiskott described three brothers with a similar disease, whose sisters were unaffected.

Author information
  • This information has been edited and peer-reviewed by contributors to the Natural Standard Research Collaboration (www.naturalstandard.com).

Bibliography
  1. Binder V, Albert MH, Kabus M, et al. The genotype of the original Wiskott phenotype. N Engl J Med. 2006 Oct 26;355(17):1790-3.
  2. Jin Y, Mazza C, Christie JR, et al. Mutations of the Wiskott-Aldrich Syndrome Protein (WASP): hotspots, effect on transcription, and translation and phenotype/genotype correlation. Blood. 2004 Dec 15;104(13):4010-9. Epub 2004 Jul 29.
  3. Natural Standard: The Authority on Integrative Medicine. .
  4. St. Jude Children's Research Hospital. Inherited Immunodeficiencies: Wiskott-Aldrich Syndrome (WAS). .
  5. U.S. Immune Deficiency Foundation. The Wiskott Aldrich Syndrome. .

Hla-b27
  • Rheumatoid disease: Researchers discovered the sequence of HLA-B27 in 1985. While researchers estimate that only 1.4-8% of the general population has the molecule, it is prevalent among patients who have rheumatoid diseases, especially ankylosing spondylitis. Although there are six subtypes of HLA-B27, there is no association between one particular subtype and inflammatory disease.
  • Ankylosing spondylitis is a rheumatic disease of the spine that causes chronic inflammation of the spine and the sacroiliac joints (located in the lower back). More than 90% of patients who have spondylitis have the HLA-B27 molecule.
  • However, even though most patients with ankylosing spondylitis have the HLA-B27 molecule, it does not mean that everyone who has the gene will develop the disease. In fact, it is estimated that up to 75% of patients who have the gene never develop clinically significant rheumatic symptoms
  • The HLA-B27 molecule is also present in about 80% of patients who have reactive arthritis. In addition, about 50% of patients who have a combination of peripheral arthritis and either psoriasis (skin disease characterized by red patches of skin covered with white scales) or inflammatory bowel disease (IBD) have the HLA-B27 molecule.
  • It remains unknown exactly how the HLA-B27 molecule triggers an inflammatory response in the body. It has been suggested that it occurs when an infectious organism that looks similar to HLA-B27 enters the body. In such cases, researchers believe that the immune system cells mistake the HLA-B27 molecule for the infectious agent. As a result, the immune system attacks itself and symptoms of rheumatoid disease occur. It has also been suggested that the HLA-B27 molecules bind to infectious agents. This may cause the immune system to attack itself. A third theory is that the HLA-B27 molecule may be closely linked to a currently unidentified gene that is responsible for triggering the immune response.
  • Acute anterior uveitis: About 50-60% of patients who have acute anterior uveitis (AAU) are HLA-B27 positive. Acute anterior uveitis (AAU) is an inflammation of the uveal tract that lines the inside of the eye behind the cornea. The incidence of this disease varies according to racial background and nationality. AAU that is associated with the HLA-B27 molecule is most common among Caucasians and least common among African Americans. Symptoms often include severe eye pain, redness near the edge of the iris (colored part of the eye), and extreme sensitivity to light.
  • While the exact relationship between the HLA-B27 gene and AAU remains unknown, several theories exist. Based on several animal studies, many cases of AAU occur after a patient develops chlamydia or an infection that causes diarrhea. Disease-causing organisms that have shown to cause AAU include Shigella, Salmonella, Klebsiella, and Yersinia species. These organisms have similar structures to the HLA molecule. As the immune system fights off these organisms, the body may mistake the HLA-B27 molecules for the disease-causing agents. When this occurs, symptoms of AAU may subsequently develop.

Hla-dr4
  • Researchers suggest that patients who have the HLA-DR molecule may have an increased risk of developing autoimmune disorders. These disorders occur when the immune mistakenly attacks the body's own tissues because they are incorrectly identified as harmful substances like bacteria or viruses.
  • The most common autoimmune disorder associated with the HLA-DR molecule is rheumatoid arthritis. This condition causes the joints to become swollen and painful. Without treatment, the pain may make it difficult for the patient to perform normal daily activities such as walking.
  • The HLA-DR4 gene has also been associated with Lyme disease. Lyme disease is caused by bacteria called Borrelia burgdorferi that are transmitted to humans via deer ticks. Lyme disease typically causes joint pain, inflammation, and arthritis.
  • Patients who have severe cases of Lyme disease and do not respond well to the antibiotic treatment are more likely to have the HLA-DR4 molecule. It has been suggested that once the disease-causing organism moves to the joints, the immune system mistakes the body cells containing the HLA-DR4 molecule for the bacteria. This consequently causes an autoimmune reaction.

Histocompatibility antigen test
  • A histocompatibility antigen blood test analyzes the HLA proteins on the surface of cells. Certain HLA proteins have been associated with specific disease. For instance, an HLA-B27 positive individual is about 87 times more likely to develop ankylosing spondylitis than someone who does not have the gene. Therefore, the test may help determine if an individual is at risk for certain diseases.
  • The test is most often used to identify good matches for tissue grafts and organ transplants. To help prevent serious complications, such as graft-versus-host disease or transplant rejection, the potential donor and recipient must be tested to determine whether their HLA molecules are closely matched making them compatible. Each person has unique HLA molecules (except for twins, who have identical molecules). Therefore, this test cannot prevent 100% of complications. However, the test can significantly reduce the number of patients who develop complications after transplantations.

Copyright © 2011 Natural Standard (www.naturalstandard.com)


The information in this monograph is intended for informational purposes only, and is meant to help users better understand health concerns. Information is based on review of scientific research data, historical practice patterns, and clinical experience. This information should not be interpreted as specific medical advice. Users should consult with a qualified healthcare provider for specific questions regarding therapies, diagnosis and/or health conditions, prior to making therapeutic decisions.

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