advised for IVIg to be approved. X-linked agamma/hypogammaglobulinaemia The patient has a confirmed or suspected Severe combined diagnosis of primary immunodeficiency immunodeficiency that must be advised. Wiskott-Aldrich syndrome X-linked lymphoproliferative syndrome Hyper IgM syndrome Severe T-cell immunodeficiency Where a diagnosis has initially been If the diagnosis is not confirmed at the first suspected, confirmation required for access dose, the confirmed diagnosis must be updated to continuing Ig therapy. prior to further Ig treatment. (A) Common variable immune deficiency - based The diagnostic criteria for CVID from the on ESID 2014 criteria European Society for Immunodeficiency Diseases have been used as the eligibility The patient is older than four years of age criteria for access to long term Ig therapy in CVID. It was acknowledged that some current at diagnosis (although symptoms may patients may require to be ‘grandfathered’ but present earlier). that moving forwards – the international diagnostic criteria should be applied. AND (A) There is evidence of a marked decrease of For patients not meeting the full criteria, a IgG and a marked decrease of IgA further indication has been developed to (measured at least twice and less than the support initial treatment with review. normal reference range for age) with or without low IgM levels and causes of secondary hypogammaglobulinaemia have been excluded AND
advised for IVIg to be approved. The patient has a confirmed or suspected diagnosis of primary immunodeficiency that must be advised. Where a diagnosis has initially been suspected, confirmation required for access to continuing Ig therapy. Common variable immune deficiency - based on ESID 2014 criteria The patient is older than four years of age at diagnosis (although symptoms may present earlier). AND There is evidence of a marked decrease of IgG and a marked decrease of IgA (measured at least twice and less than the normal reference range for age) with or without low IgM levels and causes of secondary hypogammaglobulinaemia have been excluded AND X-linked agamma/hypogammaglobulinaemia Severe combined immunodeficiency Wiskott-Aldrich syndrome X-linked lymphoproliferative syndrome Hyper IgM syndrome Severe T-cell immunodeficiency If the diagnosis is not confirmed at the first dose, the confirmed diagnosis must be updated prior to further Ig treatment. (A) The diagnostic criteria for CVID from the European Society for Immunodeficiency Diseases have been used as the eligibility criteria for access to long term Ig therapy in CVID. It was acknowledged that some current patients may require to be ‘grandfathered’ but that moving forwards – the international diagnostic criteria should be applied. (A) For patients not meeting the full criteria, a further indication has been developed to support initial treatment with review.
primary-immunodeficiency-diseases%28pid%29-with-antibody-deficiency_0.DOCX