X-linked severe congenital neutropenia

A severe congenital neutropenia that has_material_basis_in hemizygous activating mutation in WAS on chromosome Xp11.23.

Signs and symptoms

• Inverted CD4:CD8 ratio
• Recurrent bacterial infections
• Decreased total neutrophil count
• Decreased total monocyte count
• Bone marrow arrest at the promyelocytic stage
• Eczematoid dermatitis

Also known as: SCNX; XLN