A3: Immunodeficiencies Flashcards
What is the difference between primary immuno and secondary immuno
Primary are genetic defects in immune system
Secondary was injtially intact but other environmental or infective factors compromised
Are pid germline or somatic
Both but most lilely hereditary so germline
Give some causes of SID
Meds wg chemo Infections like hiv Barrier defects Malnutrition Splenectomy (removal)
How would splenectomy cause sid
Because it is a secokdary lymph tissue where adaptive response occurs
How can premature births cause sid
Botn before the igg maternal transfer
Give an example of breaxh of barriers
Eczema
Are immune deficinecies always loss of the immjne response
No can become hupersctive eg autoimmune responses, hyper allergenic
Give some consequences of immunodeficiencies
Recurrent infection Hypoallergenic (no immune tolerance) Auto inflammation Autoimmunity eg type 1 Cancer
How can metastasis occur through immunodeficiency
Because lack or tumour surveillance if lacking immune system
What are the signs of immunodeficiencies in patiwnts
Recurring infection, persistent after treatment, serious eg meningitis, unusual infection, fwmily history, incestual marriage
What is an example of an unusual pathogen
Pneumocystis jurovecii
What type of immune factors are affected in immunodeficiency
Phagocutes,complement, t cell, b frll, antibodies, combined B and T
What PID which affects neutrophils is there
Chronic granulomatous disease eg x linked
How does cgd affect neutropjils via genetic defect
Inability to produce ROS to enduce phsgocytic killing
What effects does cgd have
Infection susceptibilitybeg pneumonia
Also inflammation eg in lung, colitis
What SID fsctors affect neutrophils
Neutropenia from large infection
Meds
Malnutrition eg b12, folate
Hypersplenism
How does hypersplenism affect neutrophils
Drains blood too fast and large amlunts wnter spleen causinf neutropenia
Why is humonal immunitt defects bad in mucosa
Because larhe amounts of eg iga loss so mucosal infections like pneumonia occur
Which immunodeficiency can cause sepsis, meningitis by increased entry of pathogens
Humoral
What is the larhest PID affecting humoral immunity and what is it
X linked a gammaglobulinaemia
Low/absent ig and b cells but notmal t cells
Why is x linked a gammaglobulinaemia developed after 6 months of age
Because you get maternal transfer from mother igg
What are the 3 main reasons for SID humoral defects / hypogammaglobulinaemia/low iga/low b
Meds
Loss of proteins eg nephrotic syndrome
Malnutrition
What autoimmune disorders due to complement deficiency
SLE
Which infections are due ti lack of C3
Pneumococcal and h infleunza
What makes you susceptible to neisseria
C5,6,7,8,9 deficiency
What is the major SID causing complement c5 deficinecy
Monoantibody treatments which block c5 but ised for treatment for disorders
Which instant treatmnet can you het for immunodeficiencies
Antibiotic or antifungal prophylaxis
What are the serious treatments
Ig replacement
HSC transpant
Which immunodeficiency illness causes lack of t and b cells
Severe combined immunodeficiency SCID
Why do b cells sometimes see notmal in scid
They sre but arent ever sctivayed if no t cells
Which vaccine can scid not have
Live vaccines
What effects does scie have
Recurrent infections eg candida
Peristajr viral infections: rsv, rota
Chronic diarrhoea
Death
What do you rule out if someone has persistsnt lymphopenia
Scid
Which infection/SID causes combined immunodeficiency
Hiv, it infects cd4 cells
Other than infection, what causes combined immunodeficiency
Meds, immunotherapy, MA use
Which therapies mimic immunodeficiency to treat other things like cancer and how
Usually moAbs use by blocking or destroying immune cells
Why sre recombinant ab usually better (humanised mab inserted into vectors then eg into mouse)
Less immunogenic
