Definition:
Wiskott-Aldrich syndrome otherwise known as the eczema-thrombocytopenia-immunodeficiency syndrome (Aldrich RA, Steinberg AG, Campbell DC (1954). "Pedigree demonstrating a sex-linked recessive condition characterized by draining ears, eczematoid dermatitis and bloody diarrhea". Pediatrics 13 (2): 133-9) is a x-linked recessive disease that is characterized by eczema, thrombocytopenia of low platelet counts, immune deficiency and blood in diarrhea.
Diagnosis:
Diagnosis is usually bases on clinical parameters especially in the blood film and low immunoglobulin levels. Usually the IgM levels for person with this condition is low and the IgA and IgE are elevated and paraproteins are observed (Radl J, Dooren LH, Morell A, Skvaril F, Vossen JM, Uittenbogaart CH (1976). "Immunoglobulins and transient paraproteins in sera of patients with the Wiskott-Aldrich syndrome: a follow-up study". Clin. Exp. Immunol. 25 (2): 256-63).
Treatment:
Treatment of Wiskott-Aldrich syndrome would vary and greatly depends on the symptoms. However, in cases where there are low platelet counts, a splenectomy or platelet transfusion and blood transfusion for anemia is advised. Also intravenous immunoglobulins or IVIG are advised to boost the immune system of the person.
Symptoms and Signs:
Its symptoms includes bruising thrombocytopenia or low platelet counts, small platelet size on bloody film, eczema, recurrent infections and possibility for autoimmune disorders and malignancies.
Causes:
It is said to be inherited immune disorder caused by mutations in a gene that primarily affects the immune system.
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