Wiskott-Aldrich
Syndrome
Features:
Triad
of symptoms:
Decreased
number of delta and alpha granules
No
albinism
Severe eczema
Recurrent infections
Life-threatening thrombocytopenia
Primarily
seen in males
Decreased aggregation response to ADP, collagen, and epinephrine
Normal
aggregation response to thrombin
Note; this is typical aggregation response in
all storage
pool deficiencies