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Intravenous augmentation therapy with alpha-1-antiprotease for severe AAT deficiency*

Intravenous augmentation therapy with alpha-1-antiprotease for severe AAT deficiency*
Pretreatment testing
  • Spirometry with DLCO.
  • Liver function tests.
  • Serum IgA level.
Protocol for augmentation therapy
  • Product: Pooled plasma-derived alpha-1-antiprotease (Aralast NP, Prolastin-C, Zemaira, and Glassia).
  • Interval: Weekly.Δ
  • Patient's weight: ____kg.
  • Calculate patient's dose: 60 mg × weight in kg = _____weekly dose.
  • Aralast NP, Prolastin-C, and Zemaira are supplied as lyophilized preparations and require reconstitution according to the package insert. After reconstitution, pooled AAT should be used within three hours.
  • Infusion rate depends on specific products used and ranges from ≤0.2 mL/kg/minute to 0.08 mL/kg/minute. Consult package insert. The rate can be adjusted, if needed for patient comfort.
Supportive care
  • Cessation of smoking and avoidance of passive smoke exposure.
  • Avoidance of respiratory irritants.
  • Pulmonary rehabilitation if reduced exercise capacity.
  • Oxygen therapy (as needed).
  • Nutritional support as appropriate to maintain healthy body weight.
  • Influenza and pneumococcal vaccinations.
  • Treatment of respiratory infections (eg, influenza, bacterial bronchitis, flares of bronchiectasis, pneumonia).
  • Inhaled bronchodilators and glucocorticoids per guidelines for COPD.
  • Vaccination against hepatitis A and B viruses, if not already immune.◊
AAT: alpha-1 antitrypsin; DLCO: diffusing capacity for carbon monoxide.
* An AAT serum level <11 micromol/L or <57 mg/dL is considered severe deficiency and is usually associated with a genetic variant, such as PI*ZZ or PI*Null. For information about selection of patients for AAT augmentation, refer to UpToDate review on treatment of AAT deficiency.
¶ Infusion of alpha-1-antiprotease can be performed at home after appropriate training or in an infusion center. Epinephrine should be available, if needed.
Δ The US Food and Drug Administration approved regimen is 60 mg/kg, administered weekly. In special circumstances, 120 mg/kg is administered biweekly, or 250 mg/kg is administered monthly.
◊ Vaccination against hepatitis A and B can help prevent superimposed insults to the liver, but is not necessary prior to AAT augmentation therapy, due to the low risk of transmission.
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