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The Wiskott Aldrich Syndrome protein (WASp) is an actin polymerization regulator influencing the cytoskeleton of multiple cells. Loss-of-function mutations of WASp result in Wiskott Aldrich Syndrome (WAS) however novel mutations of WASp have been related to X-linked neutropenia (XLN). The phenotype of XLN is thought to be related to increased actin polymerization resulting in decreased neutrophil production and increased destruction. Lymphoid cells are also impacted resulting in variable immune function abnormalities. Platelet numbers are normal compared to WAS, however platelet size is variable with some large platelets. Neutropenia with other lymphocytic and immune function abnormalities leading to XLN usually cause recurrent infections early in life.