Disease or Syndrome
Wiskott-Aldrich syndrome
Subclass of:
Blood Coagulation Disorders, Inherited;
Primary immune deficiency disorder;
Genetic Diseases, X-Linked;
Hemorrhagic Disorders;
Lymphopenia
Definitions related to wiskott-aldrich syndrome:
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A rare, X-linked immunodeficiency syndrome characterized by ECZEMA; LYMPHOPENIA; and, recurrent pyogenic infection. It is seen exclusively in young boys. Typically, IMMUNOGLOBULIN M levels are low and IMMUNOGLOBULIN A and IMMUNOGLOBULIN E levels are elevated. Lymphoreticular malignancies are common.NLM Medical Subject HeadingsU.S. National Library of Medicine, 2021
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An inherited immune disorder that occurs in young boys. It causes eczema (a type of skin inflammation), a decrease in the number of platelets (blood cells that help prevent bleeding), and frequent bacterial infections. People with Wiskott-Aldrich syndrome are at increased risk of developing leukemia and lymphoma.NCI Dictionary of Cancer TermsU.S. National Cancer Institute, 2021
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(wiskott aldrich syndrome) Rare x-linked immunodeficiency syndrome of young boys characterized by eczema, thrombocytopenic purpura and recurrent pyogenic infection; IGM levels are low and IGA and IGE levels are elevated; lymphoreticular malignancies are common.CRISP ThesaurusNational Institutes of Health, 2006
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The WAS-related disorders, which include Wiskott-Aldrich syndrome, X-linked thrombocytopenia (XLT), and X-linked congenital neutropenia (XLN), are a spectrum of disorders of hematopoietic cells, with predominant defects of platelets and lymphocytes caused by pathogenic variants in WAS. WAS-related disorders usually present in infancy. Affected males have thrombocytopenia with intermittent mucosal bleeding, bloody diarrhea, and intermittent or chronic petechiae and purpura; eczema; and recurrent bacterial and viral infections, particularly of the ear. At least 40% of those who survive the early complications develop one or more autoimmune conditions including hemolytic anemia, immune thrombocytopenic purpura, immune-mediated neutropenia, rheumatoid arthritis, vasculitis, and immune-mediated damage to the kidneys and liver. Individuals with a WAS-related disorder, particularly those who have been exposed to Epstein-Barr virus (EBV), are at increased risk of developing lymphomas, which often occur in unusual, extranodal locations including the brain, lung, or gastrointestinal tract. Males with XLT have thrombocytopenia with small platelets; other complications of Wiskott-Aldrich syndrome, including eczema and immune dysfunction, are usually mild or absent. Males with XLN have congenital neutropenia, myeloid dysplasia, and lymphoid cell abnormalities.GeneReviewsUniversity of Washington, 2021
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Wiskott-Aldrich syndrome results from a combined B- and T-cell defect and is characterized by recurrent infection, eczema, and thrombocytopenia. Wiskott-Aldrich syndrome is a primary immunodeficiency disorder that involves combined humoral and cellular immunity deficiencies.Merck & Co., Inc., 2020
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Wiskott-Aldrich syndrome (WAS) is a condition with variable expression, but commonly includes immunoglobulin M (IgM) deficiency. WAS always causes persistent thrombocytopenia and, in its complete form, also causes small platelets, atopy, cellular and humoral immunodeficiency, and an increased risk of autoimmune disease and hematologic...WebMD, 2019
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Wiskott Aldrich syndrome (WAS) is a disease with immunological deficiency and reduced ability to form blood clots. Signs and symptoms include easy bruising or bleeding due to a decrease in the number and size of platelets; susceptibility to infections and to immune and inflammatory disorders; and an increased risk for some cancers (such...National Center for Advancing Translational Sciences
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Wiskott-Aldrich syndrome (WAS) is an X-linked recessive condition and is characterized by the clinical triad of thrombocytopenia, eczema, and recurrent infections. Thrombocytopenia, with low platelet volume, is present from birth and is universal. Other features manifest over time and are variable, particularly immunodeficiency, which...Athenahealth, Inc., 2019
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