Conditions treated by the Immunology department at Great Ormond Street Hospital include:
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
-
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)
-
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
- autoimmune lymphoproliferative syndrome
Management guidelines
A range of management guidelines have been prepared by the UK-Primary Immunodeficiency Network.