Acquired hypogammaglobulinaemia secondary to haematological malignancies, or post-haemopoietic stem cell transplantation (HSCT)

Version: 3.1
Published: 20 October 2018
Condition for which Ig has an established therapeutic role.

Serum IgG to be measured on two separate occasions (at least one hour apart and at least one sample taken when the patient does not have an active infection). Baseline serum levels of IgA and IgM should be provided to allow assessment of immune recovery at review.
 

• Significant hypogammaglobulinaemia with serum IgG less than 4g/L (excluding paraprotein) regardless of the frequency and severity of infections
• OR
• Serum IgG (excluding paraprotein) greater than 4g/L but less than the lower limit of the age related reference range with at least one life threatening infection in the last 12 months
   
• OR
• Serum IgG (excluding paraprotein) greater than 4g/L but less than the lower limit of the age related reference range with at least two serious infections in the last six months requiring more than standard courses of antibiotics (e.g. hospitalisation, intravenous or prolonged antibiotic therapy)
   

Initial review is required within six months by a haematologist, general physician or paediatrician and ongoing reviews at least annually to assess clinical benefit.
 
In principle, Ig should be continued or renewed only if there is a demonstrated clinical benefit; therefore documentation of clinical effectiveness is necessary for continuation of Ig therapy.
 
Cessation of Ig therapy should be considered at least after each 12 months of treatment. If serum IgM and IgA levels are trending upwards and near normal, this may suggest recovery of the immune system and a trial might be considered if the patient is well. Once the patient has normal IgA and IgM levels, the IgG is also likely to be normal and a trial off therapy may be undertaken. Ig therapy should be extended as required to enable cessation of therapy in September/October, with repeat clinical and/or immunological evaluation before recommencement of therapy.
 
Antibiotic therapy may be indicated in addition to Immunoglobulin therapy.
 

Initial review is required within six months by a haematologist, immunologist, general physician, oncologist or paediatrician and ongoing reviews at least annually to assess clinical benefit.
 
In principle, Ig should be continued or renewed only if there is a demonstrated clinical benefit; therefore documentation of clinical effectiveness is necessary for continuation of Ig therapy.
 
Cessation of Ig therapy should be considered at least after each 12 months of treatment. If serum IgM and IgA levels are trending upwards and near normal, this may suggest recovery of the immune system and a trial might be considered if the patient is well. Once the patient has normal IgA and IgM levels, the IgG is also likely to be normal and a trial off therapy may be undertaken. Ig therapy should be extended as required to enable cessation of therapy in September/October, with repeat clinical and/or immunological evaluation before re-commencement of therapy.
 
Antibiotic therapy may be indicated in addition to Immunoglobulin therapy.
 
Clinical effectiveness of Ig therapy may be assessed by:
 

On review of the initial authorisation period

• Monitoring of serum immunoglobulin levels (IgG, IgA and IgM) and any history of infection
   

On review of a continuing authorisation period

• Monitoring of serum immunoglobulins (IgG, IgA and IgM) and any history of infection
   

• A trial period of cessation of IVIg for the purposes of immunological evaluation will commence in September or October or is medically contraindicated on safety grounds (such as neutropenia, immunosuppressant medication, active bronchiectasis and/or suppuratives lung disease or severe hypogammaglobulinemia) persists where no significant improvement has occurred in the underlying condition.
   

Please note: A diagnosis of bronchiectasis and/or suppurative lung disease must be consistent with the guideline of the Thoracic Society of Australia and New Zealand (Chang AB et al. 2014).
 

• Loading Dose - One loading dose of 0.4 g/kg in the first month of therapy (in addition to the maintenance dose) is permitted if the serum IgG level is <4 g/L.
• Disseminated Enterovirus dose - One dose of 2 g/kg at any stage is permitted (in addition to the maintenance dose) in the management of disseminated enterovirus infection.
• Maintenance Dose - 0.4 g/kg every 4 weeks or more frequently, to achieve IgG trough level of at least the lower limit of the age-specific serum IgG reference range. More frequent dosing to achieve IgG trough level of up to 9 g/L is permitted if chronic suppurative lung disease is not adequately controlled at an IgG trough level at the lower limit of the age-specific serum IgG reference range. A total dose of up to 1 g/kg may be given over any 4 week period.