Wiskott - Aldrich syndrome

Wiskott - Aldrich syndrome(WAS)


 is a rare X-linked recessive disease characterized by eczema, 
 thrombocytopenia, (low platelet count ),immune deficiency   
 and bloody diarrhea (secondary to the thrombocytopenia)

WAS:  involving humoral and cellular immunity 

The risk of autoimmune disorder (autoimmune hemolytic anemia AIHA), 
and malignancies (lymphoma and leukemia) increasedin this patients.

causes: The mutations in a gene of Wiskott-Aldrich syndrome protein (WASp)

WAS protein is an essential key regulator of signal transduction and actin polymerization in HSCs. As a result, defective in immune cells function (B c ell, T cell, dendritic cells (DC) and macrophages), cell migration, cytoskeletal rearrangement and immunological synapse formation is observed in Wiskott - Aldrich syndrome patients.




Ariga T. Wiskott-Aldrich syndrome; an x-linked primary immunodeficiency disease with unique and characteristic features. Allergol Int. 2012;61(2):183-9.