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University Centre for Primary Immunodeficiencies
Department of Clinical Immunology and Allergology
St. Anne University Hospital, Masaryk UniversityBrno, Czech Republic
Anti-IgA antibodies:
Risks and safety of immunoglobulin substitution
Vojtech Thon
anti-IgA antibodies
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Anti-IgA antibodies
IgA deficiency (IgAD) and CVID
• Anti-IgA antibodies- incidence- relevance- detection- clinical consequence and therapeutic strategy
• IgG substitution therapy (IVIG, SCIG)
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Incidence of anti-IgA antibodies in Czech hypogammaglobulinemic patients
Specific anti-IgA antibodies may cause an anaphylactic reaction
following an injection or infusion of products containing IgA.
Screening, quantitative, specific and sensitive anti-IgA ELISA assay was established.
anti-IgA antibodies
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DiagnosisIgA
serum level (g/l)
Number of
patients
Patients with anti-IgA Abs
n %
IgAD< 0.01 112 15 13.4
0.01-0.05 18 2 11.1
> 0.05-0.5 18 0 0
Total 148 17 11.5%
Anti-IgA antibodies in Czech IgA deficient patients(first results)
anti-IgA antibodies
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Anti-IgA antibodies in Czech CVID patients
Diagnosis
IgA serum
level (g/l)
Number of
patients
Patients with anti-IgA Abs
n %
CVID
< 0.01 28 3 10.7
0.01-0.05 8 0 0
> 0.05-0.5 7 0 0
Total 43 3 7 %
anti-IgA antibodies
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PATIENT: Sex, date of birth, region of residence DIAGNOSIS: Date of diagnosis, onset of symptoms, disease/diagnosisTHERAPY: Date, drug, dose per weight, dose interval, route (oral / SC
/ IV), from, until, side-effects, reason stopped, compliance QUALITY OF LIFE: Days in hospital, days missed at school, days missed at
work, body weight, body heightLABORATORY: Date, time, label, value (IgG, IgA, IgM, CD3, CD4, CD8,
CD19 or CD20, CD56, Leukocytes, Thrombocytes, Erythrocytes, Lymphocytes, Granulocytes, Hb, Eosinophils, Basophils, Macrophages) in percent or/and in absolute values
+ Anti IgA Abs (red field)
EUROPEAN SOCIETY FOR IMMUNODEFICIENCIES The ESID Online Patient- & Research Database
The red fields…
www.esid-registry.org
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THE SPECIFICITY OF DETECTIONAND QUANTIFICATION (ELISA)
• TITRATION
• INHIBITION TEST– Reaction of positive serum
with positive and negative pool
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Anti-IgA Abs were found in patients
with low level of serum IgA (< 0.05 g/l, resp. < 0.01 g/l).
anti-IgA antibodies
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Therapeutic strategyanti-IgA antibodies
Investigation of anti-IgA antibodies in German CVID
(University of Freiburg, Germany and Masaryk University, Brno, Czech Republic)
German CVID: n = 88
anti-IgA antibodies were found in 8 patients (9.1 %)
Interestingly, two of the eight patients showed heterozygous mutations in TNFRSF13b/TACI.
Clinical correlation: Five positive patients had a history of anaphylactoid reactions to IVIG
and anti-IgA Ab Titer: 1 : 400 – 1 : 6400.
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et al.
Clin Immunol. 2007;122:156-62
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Horn, Thon et al., Clin Immunol. 2007
Lack of IgA+ B cellsin CVID patients with anti-IgA antibodies
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CVID: IgA serum levels correspond to paucity of IgA+ B cells
Horn, Thon et al., Clin Immunol. 2007
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CVID:IVIG change to SCIG substitution:
development of anti-IgA antibodies
Horn, Thon et al., Clin Immunol. 2007
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In a cohort of 279 hypogammaglobulinemic patients (IgAD, CVID) from the Czech and German population we found 10 % positive with anti-IgA antibodies (28 positive patients from 279 investigated).
Anti-IgA Abs were found in patients with low level of serum IgA (< 0.05 g/l, resp. < 0.01 g/l) and paucity of IgA positive B cells.
The occurrence of anti-IgA Abs in the middle European population is similar to the Scandinavian population (e.g. Koskinen et al.)
anti-IgA antibodies
Conclusion
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SCIG and IVIG
In terms of therapeutic management of CVID
patients with low serum IgA,
lacking IgA positive B cells and with anti-IgA
antibodies we recomend SCIG therapy
as a safe therapeutic alternative to IVIG
to prevent anaphylactoid reactions.
Conclusion
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Collaboration
University Hospital Freiburg, Germany and Royal Free Hospital, London
J. Horn, B. Grimbacher, U. Salzer, K. Warnatz, M. Schlesier, H. H. Peter
anti-IgA antibodies
University Centre for Primary Immunodeficiencies, Masaryk University, St. Anne University Hospital, Brno, Czech Republic
V. Thon, J. Litzman, D. Bartonkova, Z. Travnickova, J. Lokaj
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Thank you for your attention.
anti-IgA antibodies