Wiskott-Aldrich syndrome

An X-linked immune deficiency syndrome whose hallmarks are decreased resistance to infection, eczema, and thrombocytopenia. The number of T lymphocytes in the blood and lymph nodes declines, blood levels of immunoglobulin M class antibodies are reduced, and the response to many antigens is inadequate. If bone marrow transplant is unsuccessful, the patients die at a young age from infection.


An uncommon genetic disorder inherited through the X chromosome, characterized by an atypical immune system and diminished blood clotting capacity, is identified as an X-linked recessive disease. This medical condition is closely linked to various health conditions such as the presence of bloody diarrhea, eczema, immune deficiency, and thrombocytopenia, which is a decreased level of platelets in the blood.


This is a genetic condition passed down through generations where the immune system doesn’t work properly, resulting in a reduced number of platelets (cells crucial for blood clotting). It’s inherited through an X-linked recessive pattern, leading to the disease in males. Females might not show any symptoms but can transmit the gene to their offspring.


Common characteristics of this condition can encompass recurring infections (resulting from weakened immunity), a higher susceptibility to bleeding (attributable to a decreased platelet count), and the presence of eczema. Typically, symptoms become evident shortly after birth or within the first year of life. Individuals affected by this condition also face an elevated risk of developing cancers like lymphoma and leukemia, often during their adolescent or early adult years.


The goal of treatment is to alleviate the symptoms of the condition.


 


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