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8 Principles Use Of IVIG > Principles Of Immunoglobulin Therapy > Flashcards

Flashcards in Principles Of Immunoglobulin Therapy Deck (20)
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1

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

2

PI and PIDD stand for

Primary immunodeficiency and primary immunodeficiency diseases

3

Describe how to diagnose PI

Present with low total levels of IgG or normal level IgG with documented specific antibody deficiency

4

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

5

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)

6

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

7

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

8

How do you choose the route of administration for immunoglobulin

Should be patient specific. Intravenous appropriate for most patients and subset receive subcutaneous

9

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.

10

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

11

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

12

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

13

What is maximum dose of IVIG studied

Doses greater than 800mg/kg have not been rigorously studied

14

For patients with zero IgG level at diagnosis, what is the level to aim for?

IgG of 500mg/dl, preferably over 800mg/dl

15

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

16

When to give IV or SC immunoglobulin

No specific data. Clinical judgement. SC given more often; get option for poor venous access and for patients who experience severe AE

17

How many IVIG products are available and what are the differences

18 treat PI, 20 on market in U.S. (other indications such as immune thrombocytopenia)
Differences include osmolality, stabilizer, amount of IgA, IV or SC, concentrations available, grams per bottle

18

Immunoglobulin is necessary and lifesaving therapy for the following conditions

XLA (x-linked agammaglobulinemia) and CVID (common variable immunodeficiency)

19

What are controversies surrounding IG therapy are

Need for better laboratory assays of functional antibody responses, better clinical and microbiological evaluation and characterization of the recurrent infections seen in antibody-deficient patients

20

Name 6 phenotypes of PI

1. Agammaglobulinemia due to absence of B cells
2. Hypogamnaglobulinemia with poor antibody function
3. Normal immunoglobulins with poor antibody function
4. Hypogamnaglobulinemia with normal antibody function
5. Isolated IgG subclass deficiency with recurrent infections
6. Recurrent infections due to a complex immune mechanism related to a genetically defined PI disease