immunodeficiency Flashcards
innate vs adaptive immune ssytem
innate immune system: physical and chemical barrier cells - phagocytes, dendirtic cells, NK cells receptors complement system cytokines inflammation
adaptive immune system:
T cells (helper/cytotoxic)
b cells: immunoglobulin IgM, IgG, IgA, IgE
primary immunodeficiency (symptoms)
family history months on antibiotics recurrent infections e.g ear, respiratory, sinus, deep seated pneumonia deep skin abssess persistent thrush failure to thrive
what is secondary immunodeficiency?
can result from a wide array of disease processes. increases susceptibility to infection, malingnacy and autoimmune disease
chronic imbalance in hormones, metabolism, nutrients or waste product
neutrophil dysfunction secondary to hyperglycaemia predisposes bacterial and fungal infection
poor peripheral circulation leads to impaired delivery of neutrophils
infection and radiation causing SCID
Radiation: DNA damage, induces apoptosis, B cells sensitive
HIV, Malaria, herpes virus Measles: global immune suppression leading to bacterial superinfection
diagnosis of SCID
should be suspected in newborn if +ve result of a newborn screening test, recurrent resp tract infections, recurrent diarrhoea, failure to thrive, oral thursh, recurrent viral infections
lymphocyte count <300mm3
absence of thymic shadow is indicitive
minimal CD8+
hyper immunoglobulin E (IgE) syndrome
Job syndrome
associated with staphylococcus aureus infection
coarse sacies staph abscesses retained primary teet increased IgE derm problems (e.g. severe eczema)
hyper immunoglobulin E
(Job syndrome)
(Buckley syndrome)
autosomal dominant/recessive
recurrent respiratory infections (staphylococcus / haemophilus), chronic eczematoid eruptions, cold abscess and sometimes mucocutaenous candidiasis
coarse facial features
peculiarity of teeth (Fail to shed their primary teeth so have two sets simultaneously)
serum polyclonal IgE is markedly elevated
leukocytes are normal, mild eosinophilia
