Conditions treated by the Immunology department

Severe immunodeficiencies for which stem cell transplantation or gene therapy may be appropriate

• severe combined immunodeficiency (SCID) (all forms)*

• other (as yet molecularly undefined) combined immunodeficiencies

• X-linked hyper-IgM syndrome (CD40 ligand deficiency)

• Wiskott Aldrich syndrome

• X-linked lymphoproliferative syndrome

• haemophagocytic lymphohistiocytosis

• chronic granulomatous disease

• Chediak-Higashi syndrome

• leucocyte adhesion deficiencies 

*All infants affected by SCID require corrective therapy. For all other disorders each child is individually assessed regarding the need for transplantation/gene therapy.

Primary immunodeficiencies not requiring transplantation

• common variable immunodeficiency (CVID)

• X-linked agammaglobulinemia (XLA)

• transient hypogammaglobulinemia of infancy

• selective IgA deficiency

• IgG subclass deficiency (usually associated with IgA deficiency)

• C1 esterase inhibitor deficiency (hereditary angioedema)

• hyper-IgE syndrome (Job’s syndrome)

• other neutrophil disorders

• ataxia telangiectasia

Other immunological disorders

• periodic fever syndromes

• vasculitis

• autoimmune lymphoproliferative syndrome

Management guidelines

A range of management guidelines have been prepared by the UK-Primary Immunodeficiency Network.