Transfusion medicine handbook

5. Fractionated Products

5.4.14 HIZENTRA NZ / HIZENTRA

Hizentra NZ is produced from New Zealand plasma. This is available as a 4 g vial (and potentially as 2 g vial).

Hizentra, is a commercial product produced from paid donor plasma. This is available as a 4 g vial. 

Indications for use

Replacement therapy in adults and children in:

• Primary Immunodeficiency Disease (PID) and
• Symptomatic hypogammaglobulinaemia secondary to underlying disease or treatment.

Immunomodulatory therapy:

• Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) as maintenance therapy in adults and children to prevent relapse of neuromuscular disability and impairment.

Dose and Administration

The dose and regimen depend on the indication. There may also be special considerations for the elderly and paediatric patients. The following dose regimens are given as a guideline.

Replacement therapy

The dose may need to be individualised for each patient dependent on the clinical response and serum IgG trough levels.

The dose regimen using the subcutaneous route should achieve a sustained level of IgG. A loading dose of at least 0.2 to 0.5 g/kg body weight (1.0 to 2.5 mL/kg) may be required. This may need to be divided over several days. After steady state IgG levels have been attained, maintenance doses are administered at repeated intervals, from daily to once every two weeks, to reach a cumulative monthly dose of the order of 0.4 to 0.8 g/kg body weight (2.0 to 4.0 mL/kg). Trough levels should be measured and assessed in conjunction with the patient’s clinical response. Depending on the clinical response (e.g. infection rate), adjustment of the dose and/or the dose interval may be considered in order to aim for higher trough levels.

Immunomodulatory therapy in CIDP patients

The therapy with Hizentra is initiated 1 week after the last IVIg infusion. The recommended subcutaneous dose is 0.2 to 0.4 g/kg body weight per week. The initial subcutaneous dose may be a 1:1 conversion from the previous IVIg dose (calculated as weekly dose) but should not be less than 0.2 g/kg body weight per week. The weekly dose can be divided into smaller doses and administered by the desired number of times per week. For dosing every two weeks, double the weekly Hizentra dose.

The dose may need to be adapted to achieve the desired clinical response. The patient’s individual clinical response should be the primary consideration in dose adjustment.

Contraindications

Hizentra is contraindicated in patients with a history of severe systemic hypersensitivity or anaphylactic reactions/anaphylaxis to the active substance or to any of the excipients of Hizentra.

Hizentra is contraindicated in patients with hyperprolinaemia type I or II.

Precautions

Administration. Hizentra is for subcutaneous use only. If Hizentra is accidentally administered into a blood vessel, patients could develop shock. The recommended infusion rate should be adhered to. Patients should be closely monitored and carefully observed for any adverse events throughout the infusion period.

Hypersensitivity / Anaphylaxis

Embolic and thrombotic events

Aseptic Meningitis Syndrome (AMS). The syndrome usually begins within several hours to 2 days following IgG treatment. AMS is characterised by: severe headache, neck stiffness, drowsiness, fever, photophobia, nausea, and vomiting. Patients exhibiting signs and symptoms of AMS should receive a thorough neurological examination, including cerebrospinal fluid studies, to rule out other causes of meningitis.

Adverse Reactions

These are seen very commonly (≥1/10):

• Nasopharyngitis
• Headache
• Rash

These are seen commonly (≥1/100 to <1/10):

• Hypersensitivity
• Dizziness, migraine
• Hypertension
• Diarrhoea, abdominal pain
• Nausea, vomiting
• Pruritus, urticarial
• Musculoskeletal pain, arthralgia
• Fatigue, pyrexia
• Chest pain, influenza like illness, pain

Interactions with Other Medicines

Immunoglobulin administration may impair for a period of at least 6 weeks and up to 3 months the efficacy of live attenuated virus vaccines such as measles, rubella, mumps and varicella. After administration of this medicinal product, an interval of 3 months should elapse before vaccination with live attenuated virus vaccines. In the case of measles, this impairment may persist for up to 1 year. Therefore, patients receiving measles vaccine should have their antibody status checked. Refer to the Immunisation Handbook for clinical practice recommendations.