ICL 5.1: Immunocompromised Hosts Flashcards
(29 cards)
what are the 3 ways you can become immunodeficient?
- iatrogenic = due to other stuff like chemo, immune modulators, transplant, HIV
- acquired = HIV
- congenital = x-liked SCID, HIV
what clues would make you suspect an immunocompromised host?
- frequent or severe infections
2. unusual/opportunistic infections
which conditions are congenital immunodeficiencies?
- Job’s syndrome
symptoms may present in the first few weeks of life
- antibody deficiency states
symptoms usually present after maternal antibodies wane (>6 months age)
- CVID
antibody deficiency states may not manifest til adulthood
congenital immunodeficiency usually presents in the first few years of life (but not always)
what’s the list of common congenital abnormalities of the immune system?
- Job’s syndrome
- Wiskott-Aldrich syndrome
- Common variable immunodeficiency
- Chronic granulomatous disease
- Chediak Higashi syndrome
- Leukocyte adhesion deficiency
- Complement deficiency
what is Job’s syndrome?
aka Hyperimmune E Recurrent Infection Syndrome
autosomal dominant
it’s a mutation in STAT3 gene product (17q21)
what are the clinical symptoms of job’s syndrome?
present within days/months after birth with severe eczema
mucocutaneous candidiasis, recurrent sinusitis/pulmonary infections, recurrent skin infections (S. aureus)
characteristic faces…
increased level of IgE
how do you treat Job’s syndrome?
symptom control
what is wiskott-aldrich syndrome?
x-linked
mutation in WASp (Wiskott – Aldrich Syndrome protein)
what are the clinical manifestations of wiskott-aldrich syndrome?
thrombocytopenia, small platelets, neutropenia = petechiae
eczema
autoimmune diseases or malignancy/myelodysplasia
infections from encapsulated organisms
molluscum contagiosum = bumps on the skin
what is chediak-higaski syndrome?
liposomal transport protein defect (LYST)
autosomal recessive
what are the clinical manifestations of chediak-higaski syndrome?
recurrent bacterial infections
partial oculocutaneous albinism with photophobia and nystagmus
silver hair!
giant neutrophil granules
what is the treatment for chediak-higaski syndrome?
BM transplant
what is LAD1?
Deficiency of the integrin component of CD 18
autosomal recessive
with severe disease, can see delayed stump separation of umbilial cord, abnormalities of dentition, recurrent SSTI (S. aureus and Pseudomonas)
infections of the skin are often necrotizing and ulcerative, but without neutrophils on biopsy = NO PUS
what is chronic granulomatous disease?
deficiency in NADPH oxidase = no respiratory burst/H2O2 production in phagocytes
x-linked or autosomal recessive
what are the symptoms of chronic granulomatous disease?
recurrent, severe episodes of infection
S. aureus, Burkholderia, Serratia, Nocardia, Aspergillus
how do you diagnose chronic granulomatous disease?
Assay of H2O2 production
Nitroblue tetrazolium reduction test
what is CVID?
primary immunodeficiency with impaired B-cell differentiation and abnormal antibody production
it’s an “umbrella” term for a large group of genetic abnormalities (both congenital and acquired)
what are the symptoms of CVID?
Decreased concentrations of IgG, IgA, and/or IgM
poor response to vaccinations
often associated with autoimmune diseases
what do complement deficiencies lead to?
bacterial infections
what do C1-C4 deficiencies cause?
- pneumonia
2. auto-immune diseases
what do C5-C9 deficiencies cause?
N. meningitis bacteremia and meningitis
Later onset of infectious manifestations (average age 17)
Lower mortality rate compared to N. meningitis in non-ICH
how does ethanol effect your immune system?
- reduced T cell function
- reduced macrophage function
- reduced antibody production
- impaired neutrophil chemotaxis
- increased respiratory tract infection
how does trauma effect your immune system?
- lymphopenis
- decreased CD4 count
- decreased cytokine production
- decreased nK cell activity
- decreased expression of MHCII and complement receptors
- decreased neutrophil chemotaxis, phagocytosis, respiratory burs and expression of integrins
which conditions as associated with secondary immunodeficiency?
- diabetes mellitus
- renal insufficiency
- hepatic insufficiency
- malnutrition
- pregnancy
- stress
- asplenia
- aging
