Primary Immunodeficiency Flashcards
is primary immunodeficiency common or rare?
rare
what are the two types of adaptive immune disorder for primary immunodeficiency ?
antibody deficiencies
cellular immunodeficiency
what is a sign for antibodies deficiencies ?
recurrent RTIs
what is the cause of cellular immunodeficiency ?
impaired T cell function
what are three causes of innate immune disorders?
defects in phagocyte function
complement deficiency
absence of pathogen recognition receptors
what is the cause of hereditary angioedema ?
C1- inhibitor deficiency
is an allergen involved in hereditary angioedema ?
no
what are some symptoms of hereditary angioedema ?
inflammation
local pain
non pitting tissue oedema
what is the management of hereditary angioedema?
acute management - breathing (intubation)
C1 inhibitor infusion
fresh frozen plasma
state a genetic syndrome that includes immunodeficiency ?
22q11 deletion syndrome
what causes immunodeficiency in 22q11 deletion syndrome?
thymus isn’t formed properly
what autoimmune phenomena can occur with 22q11 deletion syndrome?
anaemia/thrombocytopenia
juvenile chronic arthritis
raynauld
thyroid
what is the management for 22q11 syndrome?
immunoglobulin substitution gene therapy stem cell transplant thymus transplant genetic counselling
state some causes of secondary immunodeficiency ?
environmental - malnutrition, trauma, burns
disease - infection, diabetes, renal failure, asplenia, malignancy
iatrogenic - surgery, splenectomy, medication
state some phagocyte defects which can cause innate immune disorders?
absence of neutrophils
leukocyte adhesion defect
loss of PRR (pathogen recognition receptor)
intracellular killing
what makes immunocompromised infection different from normal infection?
severity persistence unusual organism recurrent runs in families
what cells are involved in adaptive immunity?
B and T cells
are complement molecules in adaptive or innate immunity?
innate
what are the four main components of immune defence mechanism?
B cells and antibodies
T cells
phagocytes
complement system
what type of genetic defects cause primary immunodeficiency?
single genetic defects
what is the most common primary immunodeficiency ?
predominately antibody disorders
what cell is defective in antibodies deficiencies?
B cells
what molecule does neutrophils use to attach to endothelium to move out of the blood stream?
CD11/CD18
state some clinical presentation of 22q11 syndrome?
short forehead
hooded eyelids
protuberant ears
bulbous nasal tip
