Wiskott-Aldrich Syndrome
This syndrome is inherited in an X-linked manner. This is also known as the Immunodeficiency with Thrombocytopenia, Eczema and Recurrent Infections.
Clinically, patients with this syndrome will manifest recurrent infections after 6 months of age, particularly those due to polysaccharide-containing microorganisms such as meningococcus, Hemophilus influenza and pneumococcus. So, affected patients usually have pneumonia, otitis media and meningitis.
Eczema may appear by the age of 1 year. Allergic manifestations may be present together with eczema which is most often complicated by secondary infections.
The earliest abnormalities found in affected individuals are usually bleeding as a result of thrombocytopenia (low platelet count) that may appear at birth. Platelet count may range from 5-100 thousands/mm.
There is hypogammaglobulinemia as a result of hypercatabolism of immunoglobulin. The hypogammaglobulinemic state of the patient may subsequently develop recurrent bacterial infections.