How much gammaglobulin ?
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How much gammaglobulin ?
INGID VIIth Meeting
Budapest, October 7, 2006
Rolf Gustafson
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Ökad infektionsbenägenhet
1880s Immunotherapy von Behring, Kitasato
1950s Substitution therapyBruton
1980s Immunomodulation Imbach, Barandun
Immunotherapy - a short history
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Ökad infektionsbenägenhet
Serum from immunized animals (mainly horses)
1895 Diphtheria antiserum
1908 Meningococcal antiserum
1925 Streptococcal antitoxin
Immunotherapy
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Ökad infektionsbenägenhet
1933 Placental extracts Diphteria
Scarlet fever
Polio
Measles
1935 Convalescent human serum Rheumatic fever
Impetigo
Measles
Chicken pox
Scarlet fever
Immunotherapy
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Ökad infektionsbenägenhet
1940
The US National Research Council asked Dr.
Edwin Cohn to identify a stable blood derivate
or substitute.
1941
The American Red Cross began collecting blood.
A grant of 10,000 USD for large scale
production of human albumin was given.
Substitution therapy
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Ökad infektionsbenägenhet
By help of manipulating the:
ethanol concentration
temperature
pH
Separation of plasma proteins
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Rolf Gustafson/Baxter Medical
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Rolf Gustafson/Baxter Medical
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Rolf Gustafson/Baxter Medical
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Ökad infektionsbenägenhet
WHO
International Red Cross
International Society of Transfusion (ISBT)
Council of Europe
European Union
European Agency for the Evaluation of
Medicinal Products (EMEA)
FDA
Statements, Directives & Resolutions
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IgG Trace amounts of IgM & IgA
Soluble CD4 & CD8
Soluble HLA
What is gammaglobulin ?
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Rolf Gustafson/Baxter Medical
Half - life of IgG
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0 3 6 9 12 15 18 21
IgG
%
Weeks
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Ökad infektionsbenägenhet
Gammaglobulin became available in the mid
– 1940s and was used for preventing viral
diseases like:
Measles
Hepatitis A
Gammaglobulin
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Bruton´s boy
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Ökad infektionsbenägenhet
…………. started as subcutaneous
injections at monthly intervals
Substitution therapy
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Ökad infektionsbenägenhet
1950s - 1960s
Medical Research Council in England
Data from 176 patients
25 mg / kg / week 50 mg / kg / week
fewer deaths
less cases of pneumonia
IgG 1.2 – 2.6 g/L IgG 2.9 – 3.8 g/L
Substitution therapy
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Ökad infektionsbenägenhet
Late 1980s
consencus of giving 300 - 400 mg / kg / month
IgG 4 – 5 g/L
1987
Roifman et al. reported that 600 mg / kg / month
was better than 200 mg / kg / month
IgG > 5 g / L
Substitution therapy
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Ökad infektionsbenägenhet
2006
Approved and recommended dose:
400 - 800 mg / kg / month
Substitution therapy
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Ökad infektionsbenägenhet
Evaluation
Clinical outcome
Increasing IgG level
Substitution therapy
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Ökad infektionsbenägenhet
Intravenous infusions
Substitution therapy
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Rolf Gustafson/Baxter Medical
IVIG - IgG levels
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IgG
g/L
Day
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Ökad infektionsbenägenhet
Subcutaneous infusions
Substitution therapy
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Rolf Gustafson/Baxter Medical
SCIG - IgG levels
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IgG
g/L
Days
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Ökad infektionsbenägenhet
Indications
X-linked agammaglobulinemia (XLA)
Common variable immunodeficiency (CVID)
Severe combined immunodeficiency (SCID)
Wiskott Aldrich syndrome (WAS)
Substitution therapy
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What about IgG subclass deficiences ?
In selected patients with:
IgG1 deficiency
IgG2 deficiency
IgG3 deficiency
Substitution therapy
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Rolf Gustafson/Baxter Medical
Half - life of IgG3
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0 7 14 21 28 35 42 49
IgG3
%
Days
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Ökad infektionsbenägenhet
What about selective IgA deficiency ?
In selected adult patients with:
at least 4 treatments with antibiotics / year
Substitution therapy
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Gammaglobulinbehandling
Gustafson R, Gardulf A, Granert C, Hansen S, Hammarström L
Prophylactic therapy for selective IgA deficiency. Lancet 1997;350:865
SCIG therapy in IgA deficiency
IgG therapy: 100 mg/kg/week
Infections per year Before IgG therapy During IgG therapy
0 – 1 0 5
2 – 3 0 5
≥ 4 10 0
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Ökad infektionsbenägenhet
What is the goal with patient education ?
The goal is …
that the patient becomes an active and encouraged part in
the decision-making process and have the knowledge about
self-care activities and behaviors that foster health and
health-related quality of life instead of being a passive
receiver of information.
A. Gardulf, 2002
Patient training & education
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Training & education should include knowledge &
understanding of:
primary antibody deficiencies
aim and importance of IgG therapy
infections & antibiotic treament
systemic adverse reactions
self-care and prevention
behaviour changes
self-infusion technique
Home-therapy training program – the key to success