IMMUNO Flashcards
(30 cards)
Secondary C3 defiency causes , and associations
Nephritic factor - stabilises C3 convertase (membranous glomerulonephritis type 3) - also partial lipodystrophy
RA - complement
Increased CH50
Reduced C1 inhibitor disease
hereditary angioedema, facial swelling; in severe cases the airway can become compromised
leading to respiratory distress.
Important cell in anaphylaxsis
mast cells release granules containing histamine and heparin. Histamine
causes vasodilatation leading to the characteristic features of inflammation
(oedema, warmth and redness of the skin). The ‘flare and wheal’
skin reaction is a feature of histamine release by mast cells. Mast cells
play a role in diseases such as asthma, eczema and allergic rhinitis.
IgA structure and function
monomer or a dimer (joined by a short peptide
known as the J chain). Its role is primarily related to the protection of
mucosal surfaces via salivary, respiratory, gastrointestinal and lacrimal
secretions. IgA is also present in breast milk, providing passive immunity
in neonates.
IgG
(C) is the most abundant antibody and occurs in monomer form in the circulation. The various
subclasses of IgG perform different functions, for example IgG2 is important in fighting encapsulated bacteria. IgG also has a role in activating complement proteins.
IgD
uncommon immunoglobulin in the body
and is found on the cell surface of immature B cells. IgD provides an essential role in lymphocyte activation.
Goodpasture’s syndrome, an autoimmune disease
triggered by a type II hypersensitivity reaction. It is characterized by glomerulonephritis and haemoptysis.
HLA DR2
Graves’ disease, systemic lupus erythematosus (SLE) and myasthenia gravis.
HLA DR3
type I diabetes mellitus and rheumatoid arthritis; in these diseases
HLA DR4
Coeliac hla
HLA DQ2
DC migrate to LNs via
CCR7 - ligands are CCL19 + CCL20 (these are all chemokines - other examples are IL-8, rantes, MIP-1 alpha and beta)
Most common type of SCID and enzyme deificency
then given another type of SCID
X-lined (45%) - COMMON gamma chain Xq13.1 (shared receptor for multiple interlukins e.g. IL-2, IL-4, IL-7, IL-9, IL-15, IL-21) --> cant respond to CKs --> early arrest T-cells and immature B-cells (nomral or high B-cells but no Ig) ADA def (adenosine deaminase deficiency enzyme - can tx by replacing this) - cant respond to CKs so early arrest T-cells/Nk cells --> v low or absent T-cells/NK , immature b-cells produced but also low in numbers
Most severe type of SCID
Most severe is reticular dysgenesis caused by mitochondiral energy metabolism enzyme adenylate kinease 2
unwell by 3months (as before have IgA from colostrum, IgG from placenta), diarea, failure to thrive, ulcers, GVHD (Bm colonised by maternal Lymphocytes as empty)
failure of neutrophils, inheritence, enzyme
Kostmann , AR , HCLS1-associated protein X-1 (HAX1)
Cylic neutropenia (episodic neutropenia every 4-6weeks) enzyme and inheritence
Neutrophil elastase (ELA-2) , autosomal dominant (cf innate immune deficiencies are predominantly inherited in an autosomal or X-linked recessive pattern)
Chronic granulomatous disease , inc tx
Absent resp burst (NADPH oxidase deficiency)
Mostly x-linked recessive, some AR
granuloma, Hepatosplenomgly, LYMPHADENOPATHY
IFN gamma boosts macrophage fucntion
Leukocyte adhesion defiency (problem, sx, tx)
CD18 (b2 integrin subunit) defiency + CD11B
together make LFA-1 binds ICAM-1
delayed seperation UC, delayed wound healing, chronic skin infections
BMT
CK defieincies
IFN-y + IL-12 AND THEIR RECEPTORS –> tb!! Salmonella
NK cell deficiency tx
acylovir , IFN-A stimulates NK cytotoxic function
most common inherited compleement deficienct
C2
MBL def prevalence
1/3 of people, often AS
acquired SLE effect on complement
reduces C4 + C3 (more so C4)
all the diff macrophages in tissues
histiocye, microglia, sinusodial lining cell, kupfer cell, osteoclast, mesangial cell, langerhans cell, macrophage like synoviocytes , alveolar macrophage
