primary & secondary antibody deficiency. antibodies & immunoglobulins
TRANSCRIPT
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PRIMARY & SECONDARYANTIBODY DEFICIENCY
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ANTIBODIES&
IMMUNOGLOBULINS
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PRIMARY ANTIBODY DEFICIENCY
The European internet-based patient and research database for primary immunodeficiencies: results 2006-2008.Gathman et al., Clin Exp Immunol (2009); 157 Suppl 1: 3-11.
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Brit Med J (1989); 298: 516-7
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THERAPEUTIC IMMUNOGLOBULIN
• 1970s - IMIg
• 1980s - IVIg
• 1990s - IVIg, SCIg
• 2000s - product safety- infusion rates / concentration- immunoglobulin retrieval
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REPLACEMENT THERAPY
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TREATMENT OUTCOMES
Wood et al. Clin Exp Immunol (2007); 149: 410-423
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EFFICACY & ADVERSITY
• Immunoglobulin• Excipients• Soluble CD4/ CD8/ HLA• Cytokines
Clin Exp Immunol (2004); 136: 111-3
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IVIg & SCIg
ESID Register 2009
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HOME THERAPY
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2008 and 2011
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SAME OLD SAME OLD
• Core of PID management• No alternatives• Lifelong requirement (usually)• Effective
(bacterial infection, antibiotic usage, QoL, hospitalisation, life expectancy)
• Dose requirement in:- frequent breakthrough infections- chronic inflammation / tissue damage- poor prognosis disease variants
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WHAT’S NEW?
The three Rs:
• Reorganisation• Reclassification • Aarrrgh
- ongoing uncertainties over dosing / target levels
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DOSE?
Impact of trough IgG on pneumonia incidence in primary immunodeficiency: A meta-analysis of clinical studies.Orange JS et al. Clinical Immunology (2010); 137: 21-30
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DOSE: INDIVIDUALISATION
‘The goal of replacement therapy should be to improve clinicaloutcome and not to reach a particular IgG trough level.’
J Allergy Clin Immunol (2010);125:1354-60
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DOSE: INDIVIDUALISATION
‘….individualizing the dosage….is preferable to using mean pharmacokinetic parameters.’
Clin Immunol (2011);139:133-41
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RECLASSIFICATION
• Specific Antibody Deficiency
• Kawasaki Disease ‘Other’ Section
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REORGANISATION
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PRIMARY ANTIBODY DEFICIENCY DISORDERS
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SPECIFIC DISORDERS
Thymoma with immunodeficiency(Good’s Syndrome)
Combined immunodeficiencies requiringhaemopoietic stem cell transplantation
(HSCT)
Specific antibody deficiency(SAD)
Transient hypogammaglobulinaemia of infancy(THI)
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SPECIFIC DISORDERS
DISORDER RECOMMENDATION / REQUIREMENT
GOOD’S Profound B cell depletion / significant antibody deficiency
HSCT Duration based on B cell reconstitution post-transplantation
SAD Robust application of selection criteria
THI Define planned duration of therapy prior to initiation
(GRADE C, LEVEL III)
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SUMMARY: PID
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SECONDARY ANTIBODY DEFICIENCY
ANTIBODYDEFICIENCY
PRIMARY SECONDARY
Malignant diseaseDrugsProtein-losing statesInfection (cause & effect)Systemic diseaseIatrogenic causesChromosomal abnormalities
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WHAT’S NEW?
• Secondary Antibody Deficiency
• Revision / collation into a single indication+ review outcomes (infection / hospitalisation)+ dosing (minimum IgG trough 6 g/L)
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RECOMMENDATIONS
• Irreversible hypo-• Hypo- associated with CLL/NHL/MM etc.
and
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GUIDELINES
‘Systematically developed statements to assist practitioner and patient decisions about appropriate health care for specific clinical circumstances’
• Evidence-based use• Consistency of care• Access to safe, high quality products• Security of supply• Utilising scarce resource
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OUTCOMES
COMPLICATIONSPROGRESSION OF COMPLICATIONS
QUALITY OF LIFEWORKING CAPACITYLIFE EXPECTANCYOPTIMISED GROWTH / DEVELOPMENT
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