Principles Of Immunoglobulin Therapy Flashcards
Overview of IVIG to treat Primary immunodeficiency etv
Indication to use IVIG
As replacement therapy for patients with primary immunodeficiency characterized by absent or deficient antibody production and in most cases, recurrent or unusually severe infection
PI and PIDD stand for
Primary immunodeficiency and primary immunodeficiency diseases
Describe how to diagnose PI
Present with low total levels of IgG or normal level IgG with documented specific antibody deficiency
Once diagnosed, how long will a patient stay on IVIG treatment for PI
Indicated as continuous replacement therapy for primary immunodeficiency. Treatment should not be interrupted once a definitive diagnosis has been established
What is dose of IVIG for PI
Indicated for patients with PI starting at 400-600 mg/kg every 3-4 weeks intravenously. (Subcutaneous doses are weekly or biweekly)
Is measuring IgG trough levels necessary to diagnose?
No. Trough levels can be useful to guide care yet NOT useful in many ways and should not be consideration in access to IVIG therapy
What locations can a patient go to in order to receive IVIG intravenous infusion
Hospital, outpatient hospital, community office or home based available, depends on clinical presentation of patient
How do you choose the route of administration for immunoglobulin
Should be patient specific. Intravenous appropriate for most patients and subset receive subcutaneous
Why is IVIG blood products not interchangeable
IVIG is blood product made differently by each manufacturer. Some contain sugar or high sodium so less ideal for diabetics or hypertension patients Sticking with same product is likely best for the patient experience. 2002 survey showed 34% of all infusion related AEs occur in context of a product change. It is also not generic drug.
Define PI
Primary immunodeficiencies (PI) are a group of diseases caused by inherent defects of the human immune system. These defects render the patient susceptible to infectious diseases. The infections can occur repeatedly, severely and atypically damage organs, reduce quality of life and shorten lifespan. In many of these diseases, infection is a result of a deficiency in antibody producing components of the one system
What are the 3 overarching primary immune defects that respond to IVIG treatment
Primary immune defects with absent B cells
Primary immune defects with hypogamnaglobulinemia and impaired specific antibody production
Primary immune defects with normogammaglobulinemia and impaired specific antibody production
Any patient who fits the description should receive regular IVIG therapy without interruption and without need to continually re-establish diagnosis
How often should IVIG be infused
Guidance is every 3-4 weeks yet can be shortened to improve clinical efficacy and outcomes. Using infusions greater than every 4 weeks is not recommended in any of the FDA approved products
What is maximum dose of IVIG studied
Doses greater than 800mg/kg have not been rigorously studied
For patients with zero IgG level at diagnosis, what is the level to aim for?
IgG of 500mg/dl, preferably over 800mg/dl
What adverse effects may occur during IVIG infusion
Acute thromboembolism, hypotension, seizures, aseptic meningitis syndrome, anaphylaxis, acute respiratory distress syndrome (ARDS), pulmonary edema, apnea and transfusion associated lung injury (TRALI). All IVIG carry black box warning for acute renal failure.
61% of patients have infusion related adverse events (AE). 44% had serious AEs
