Immunology Flashcards
what is Kostmann Syndrome
Severe chronic neutropenia
clinical presentation of Kostmann syndrome
Infections, usually within 2 weeks after birth
Fever
Failure to thrive
Oral ulceration
examples of opsonins
Complement C3b
IgG
CRP
what are examples of phagocyte receptors
Fc
CR1
features of Chronic granulomatous disease
recurrent deep bacterial infections recurrent fungal infections failure to thrive Lymphadenopathy and hepatosplenomegaly Granuloma formation
what is an investigation for chronic granulomatous disease
NBT test
defects in what network is linked to susceptibility to intracellular bacteria
IL 12 - gIFN
how can phagocyte recruitment be investigated
FBC
Presence of pus
Expression of neutrophil adhesion molecules
is there pus is chronic granulomatous disease
yes
what is Recricular dysgenesis
Defects of haemopoetic stem cells Failure of production of; neutrophils lymphocytes monocyte/macrophages platelets
what is the only treatment of recricular dysgenesis
bone marrow transplantation
what is severe combined
immunodeficiency
Failure of production of lymphocytes
what are abnormal presentations of SCID
unusual skin diseases
family history of early infant death
3-4 month old baby gets infection but is fine after - what disease is this
transient hypogammaglobulinaemia of infancy
what is transient hypogammaglobulinaemia of infancy
immune systems of 3-4 month old babies slow to mature
what is DiGeorge syndrome
development defect of 3rd/4th pharyngeal pouch
features of DiG syndrome
Low set ears abnormally folded ears, high forehead,
cleft palate, small mouth and jaw
Hypocalcaemia secondary to hypoparathroidism
Oesophageal atresia
T cell lymphopenia
Complex congenital heart disease
what is the IL12-gIFN network pathway
Infected macrophages stimulated to produce IL12
IL12 induces T cells to secrete gIFN
Then gIFN feeds back to macrophages and neutrophils
stimulates production of TNF
activates NADPH oxidase
what is essential investigation in T cell deficiencies
HIV test
what is Bruton’s X-linked hypogammaglobulinaemia
failure to produce mature B cells
no circulating B cells
no plasma cells
no circulating antibody after first 6 months
what are the 4 types of hypersensitivity reactions
Type I: Immediate hypersensitivity
Type II: Direct cell killing
Type III: Immune complex mediated
Type IV: Delayed type hypersensitivity
what is angioedema
Self-limited, localised swelling of subcutaneous tissues or mucous membranes
what is Spontaneous mast cell degranulation
Mast cells in the skin become very twitchy, if you touch them they degranulate.
what are non-allergic causes of Spontaneous mast cell degranulation
drugs - opiates, aspirin, NSAIDS
thyroid disease
idiopathic
physical urticaria
what is Samter’s triad
asthma, nasal polyps and salicylate sensitivity
what test is the gold standard for diagnosing allergy
skin-prick test
what are anti-histamines
H1 receptor antagoinsts
what is used in anaphylaxis and how does it work
adrenaline
Acts on B2 adrenergic receptors to constrict arterial smooth muscle
what are anaphylotoxins
Fragments of complement proteins released after activation
increase permeability of blood vessels
what are some examples of Type II hypersensitivity diseases
Goodpasture’s syndrome
Guillan Barre syndrome
Systemic lupus erythematosus (SLE)
what is acute hypersensitivity pneumonitis
Immune complexes deposited in the walls of alveoli and bronchioles
what causes the wheeze in hypersensitivity pneumonitis
inflammation of terminal bronchioles and alveoli caused by activated phagocytes and complement
what causes breathlessness in hypersensitivity pneumonitis
alveolitis caused by activated phagocytes and complement - results in decreased efficiency of gas transfer
what causes malaise, pyrexia in hypersensitivity pneumonitis
systemic manifestation of inflammatory response
what causes renal dysfunction
Deposition of IgG immune complexes
what is the mechanism of type 4 hypersensitivity
Initial sensitisation to antigen
- generation of “primed” T cells
Subsequent exposure
- activation of previously primed T cells
- recruitment of macrophages, other lymphocytes, neutrophils
- release of proteolytic enzymes, persistent inflammation
what is variolation
The same organism is being administered as the one that can cause disease, but the route of administration is different
when is there active transport of maternal IgG
3rd trimester
what does breast milk contain especially colostrum
IgA
what is IgA important for
colonisation of infant gi tract
what class do all nucleated cells express
Class I
what cells express Class II
Specialised antigen-presenting cells
acute cellular rejection mechanism
Recognition of donor antigens by CD4+ cells
Activation of CD4+ cells
Production of cytokines
Recruitment and activation of macrophages and neutrophils
Type IV hypersensitivity response
what are the symptoms of acute cellular rejection
Kidney transplant: Rise in creatinine, fluid retention, hypertension
Liver transplant: Rise in LFTs, coagulopathy
Lung transplant: breathlessness, pulmonary infiltrate
hyperacute rejection
Rapid destruction of graft within minutes-hours
Mediated by pre-formed antibodies that react with donor cells
why would an individual have preformed antibodies against donor cells
1) blood group antigens - naturally occurring preformed antibodies, bind surface molecules found on everything
2) HLA antigens - arise through previous exposure
when does hyper acute rejection occur
recipient has pre-existing anti-donor HLA antibodies
recipient has different blood group
what is the difference between hyper acute and acute vascular rejection
hyper acute the antibody is already there
vascular rejection antibody is made after transplantation
what is chronic allograft failure
Cellular proliferation of smooth muscle of vessel wall - leads to blood vessel getting smaller.
Occlusion of vessel lumen
what are side effects of immunosuppressive agents
Drug toxicity
Infection
Malignancy (cancer)
Atherosclerosis
what are examples of side effects of immunosuppressive agents
Nephrotoxicity after non-renal transplantation
Ciclosporin associated gingival hypertrophy
Form of SCID, causes skin rashes and infection across whole body
Graft versus host disease
