Immunodeficiencies Flashcards
(101 cards)
Warning signs for adult primary immunodeficiency disorders
4 or more infections requiring anitbiotics within one year - otitis, bronchtiis, sinusitis, penumonia
Recurring infections or infection requiring prologned antibiotic therapy
2 or more severe bacterial infections - osteomyelitis, menignitis, speticaemia, cellulitos
2 or more radiologically proven pneumonia within 3 yrars
INfection w unusual localisation or pathogen
PID in family
FTT in paeds
Ass features primary immunodeficiency
Atypical eczema
Chronic diarrhoea
FTT
Telangiectasia - ataxic
Hepatosplenomegaly
Endocrinopathy
Chronic osteomyeltisi/deep seated abscess
Mouth ulcers
AI
FH
Scondary antibody deficiency causes
Etrems of age
Urameia
Toxins
Acute and chronic infections
Burns - lose skin
Myotonic dystrophy
Protein-losing states
Lymphangiectasia
Examination for immunodeficiencies
Weight and heigh t- FTT
Structural damage from infections w look or scan eg ears, lungs, sinuses
AI features - vitiligo, alopecia, goitre
Absent tonsils - XLA
lymphadenopathy
Hepatosplaenomegaly
Other potnetial diagnositc features - telagniectasia, eczema
Features of immunodeficiencies
Increased susceptibility to infection
Susceptibility to cancers
Increased AI diseases
What deficiency is
First investigations for immunodeficiencies
FBC - individual white cells not just overall count
IgG, IgA, IgM
Serum electrophoresis
Lymphocyte surface markers - T cell (CD4/CD8), B cells, NK cells, B cell subclasses - naive, memory, class switched
Specific antibody responses
Later - genetics, complement AP100/CH100, neutrophil burst assay, lymphocyte prolif assay
Complement investigation
AP100/CH100
Neutrophil investigation
Neutrophil burst assays - can go oxidative burst to kill pathogens?
Lymphocyte investigations
Lymphocyte proliferation assays - if can proliferate on exposure to pathogen
Humoral deficiency presentations
Antibody deficiency
Recurrent sinopulmonary infections
Chronic GI infections
Bactaraemias
Meningitis
Como pathogens incl encapsulated bacteria
Common pathogens causing humoral deficiency
S.pnuemoniae, H.influenzae type B, N.meningitidis
Giardia, cryptosporidia, campylobacter
Conditions causing humoral (antibody) deficiency
Selective IgA def
Common variable ID
Specific AB D
XLA
X lymphoprolif disease
Hyper IgM syndrome
Hyper IgE syndroe
What immunodef is most common
Hypogammaglobulunaemia
Size of Igs
IgM - pentameter
IgA - dimer
IgG - monomer
What Ig deficient if losing through gut or kidney
IgG - monomer - smallest
When is low IgM noraml
Older age/normal variation
V low in lymphoprolif disease
How assess functioning of Igs
Response to vaccines
baseline serology
give pneumovax vaccine
assess antibody level weeks later
Use pneumococcal IgG antibodies
Which vaccines are easier to make an immune repsonse to
Proetin
Prevenar can be used if necessary for antibody respone testing but elss helpul than pneumova
How diagnose sepcific antibody deficiency
Pneomovax vaccination - below 4 x greater antibodyes 4-6 weeks later than baseline
Check at 6 months in case of loss
What measure if use conjugated pneumococcal vaccine to test for specific antibody deficinecy
Number of serotypes immune system responded to -
normal = >7 >0.35mcg/ml
Causes of reduced production of immunoglobulins
Primary ID - CVID, XLA - genetic
Medications
Malignancy - multiple myeloma, lymphoma, leukaemia
Systemic illness - sev infection -> bone marrow supression
Medications causing reduced production of immunoglobulins
Prednisolone
Azathioprine
Methotrexate
Rituximab
Anti-epileptics
Antipsychotics
Causes o increased loss of Igs
Nephrotic syndrome
Coeliac disease,
IBD
Peritonneal dialysis
How diagnoise CVID
> 4 years old
Low IgG
Low IgA+/- IgM
Poor or absent repsonse to immunisation
Exclusion of other immunodeficiencies