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Wiskott-aldrich syndrome

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Wiskott-Aldrich syndrome is a genetic disease, which compromises the immune system involving T and B lymphocytes, and blood cells that help control bleeding, platelets.

Symptoms of Wiskott-Aldrich Syndrome

Symptoms of wiskott-Aldrich syndrome can be:

Tendency to bleeding:

  • Reduced number and size of platelets in the blood; Cutaneous hemorrhages characterized by red-blue dots the size of a pin head, called "petechiae", or they may be larger and resemble bruises; Bloody stools (especially in childhood), bleeding gums and prolonged nosebleeds.

Frequent infections caused by all types of microorganisms such as:

  • Otitis media, sinusitis, pneumonia; Meningitis, pneumonia caused by Pneumocystis jiroveci; Viral infection of the skin caused by molluscum contagiosum.

Eczema:

  • Frequent infections of the skin; Dark spots on the skin.

Autoimmune manifestations:

  • Vasculitis; Hemolytic anemia; Idiopathic thrombocytopenic purpura.

The diagnosis for this disease can be made by the pediatrician after clinical observation of symptoms and specific tests. Assessing the size of platelets is one of the ways to diagnose the disease, as few diseases have this characteristic.

Treatment for Wiskott-Aldrich Syndrome

The most suitable treatment for Wiskott-Aldrich Syndrome is bone marrow transplantation. Other forms of treatment are the removal of the spleen, as this organ destroys the small amount of platelets that people with this syndrome have, the application of hemoglobin and the use of antibiotics.

Life expectancy for people with this syndrome is low, those who survive after ten years usually develop tumors such as lymphoma and leukemia.

Wiskott-aldrich syndrome