Wiskott-Aldrich syndrome (WAS) is a complex, inherited immunodeficiency disorder. It is also referred to as Wiskott disease.
Understanding Wiskott-Aldrich Syndrome (WAS)
WAS is an X-linked primary immunodeficiency, meaning it's caused by a genetic defect on the X chromosome. This primarily affects males, as they have only one X chromosome. Females, with two X chromosomes, can be carriers.
Key Characteristics of WAS:
- Rashes: Eczema is a common symptom.
- Recurrent Infections: Due to a weakened immune system.
- Low Platelet Levels (Thrombocytopenia): This is the most prominent feature, leading to bleeding problems.
Bleeding Problems in WAS:
These problems arise because the platelets are:
- Abnormally Low: Fewer platelets are present to clot blood.
- Dysfunctional: The existing platelets don't work properly, impairing the body's ability to form clots.
In Summary
Wiskott-Aldrich syndrome or Wiskott disease is an X-linked immunodeficiency characterized by eczema, recurrent infections, and thrombocytopenia, leading to significant bleeding issues.
