Immune Dis and Defic. Flashcards
In the first 2-3 years of life, what is a “healthy” # of otitis media and gastroenteritis?
More than 6 episodes of otitis media
or
More than 2 episodes of gastroenteritis/yr
4 components of immune fxn
- Anatomic
- Phagocytes
- Cellular immunity
- Humoral immunity
*there are diff tests you can run on all of these - refer to slide near end of lecture
Humoral immunity
specific ab (B cells) - Ig_
Complement
Public vs Private Immunodeficiency phenotypes
- Which one can wane with age?
- You have a broad syndrome/disease that leads you suceptible to multiple infxns w/ multiple organisms
- Private phenotype: you have a very specific mutation/selective defect that leads you suceptible to one infxn
* Private phenotype can wane with age: can develop antibodies
Most common source of immunodeficiency?
Usually secondary:
Malnutrition
- Vit A def
- Iron def
- Micronutrient def
- less caloric intake
–> increased risk for infxn and decreased outcome of infxn
2nd and 3rd largest source of immunodef.
2nd: HIV-1/AIDS
- 35 mill infected
3rd: Age
Phagocytes (WBCs) mainly consist of which two types of cells?
PMNs (60%)
- neutrophils
Lymphocytes (30%)
- 1500-4000
Monocytes (10%)
- macrophages
Sources of infxn with chemotherapy-associated Neutropenia
- Intestines
- transcytosis from epithelium
- perirectal abcess - Periodontal
- Skin/Catheters
*pts may have less inflammation (tumor, rubor, calor, dolor)
Chronic Granulomatous Disease (CGD) pts have _____ phagocyte number, but _______ fxn.
- Presentation
Sufficient phagocyte number, but decreased fxn
- Defect in NADPH oxidase (oxygen radicals decreased)
Can die young from infxn (fam hx)
*more likely to get uncommon organisms
Conductor/director of immunity
CD4+ T cells
Tells everybody what to do and when to do it. They dont actually kill themselves.
causes of decreased cellular immunity
- Malnutrition
- Oral steroids
- Immunotherapy
- Chemotherapy
- Transplantation
- HIV/AIDS
Causes of pneumonia in:
Elderly
- S. pneumoniae
- H. flu
- Influenza (Virus winter)
Causes of pneumonia in:
Young adult
- M. pneumoniae
- C. pneumoniae
- S. pneumoniae
Causes of pneumonia in:
AIDS
S. pneumoniae
P. jirovecii (PCP)
Causes of pneumonia in:
BMT
Nosocomial
SE Asia/Africa
BMT: Aspergillus sp
Nosocomial: GNR
SE Asia/Africa: TB
Protozoans you are more likely to get with impaired cellular immunity
- Toxoplasma
2. Cryptosporidium
Helminths you are more likely to get with impaired cellular immunity
Strongyloides
Viruses you are more likely to get with impaired cellular immunity
HSV
CMV
Varicella
Secondary immunodeficiecy in developing countries vs developed countries
Developing country:
- Malnutrition
- HIV-1/AIDS
- Measles
- Age
Developed country:
- Immunotherapy (steroids)
- HIV-1/AIDS
- Cancer Therapy
- Transplantation
3 pathogens with invasive polysacc capsules
- which antibody is going to recognize it?
- strep pneumo
- G. mening
- H. flu
IgG2 - recognize polysaccharides
Antigen binding region
Fab (Fragment Antigen Binding)
- variable region
- each is unique
Which region of antibody defines isotype?
Fc Constant region
- activates compliment
- binds phagocytes via Fc receptors
Antibody deficiency leaves you susceptible to
- Respiratory infxn
- Encapsulated bacteria
- Viruses (enterovirus)
- Giardia
IgA1,2 are found where?
Mucosal>blood
Hyper IgE syndrome
Job’s syndrome
Most common primary immunodefienciency
Selective IgA deficiency
- Fam hx
*normally you produce more IgA than IgG and IgM - no mas
Lots of people have this, m: there is very little associated increased infxn rate (look for concurrent infxn: like low IgG2)
Which complement stimulates opsonization/phagocytosis
C3
Complements act with antibodies to “opsonize” bacteria and facilitate lysis of GNR or killing by phagocytes
List the primary immunodefiency in adults
- IgA deficiency
- IgG2 subclass def
- CVID - common variable immunodeficiency
- Hyper-IgE syndrome (job’s syndrome)
- Cystic fibrosis
*most primary immunodeficiencies are present in childhood and a result of single gene defects
Immunodeficiency is an increased incidence and severity of what 3 things?
- INfection
- Malignancy
- Autoimmunity
How id neutropenia defined?
PMNs are usually
In the first 10 yrs, what is the healthy number of URI/yr?
less than 6-8 URI/ yr
Which complement deficiency is most common?
Which complement deficiencies presents with pyogenic infxns?
Which with serial Neisserial infections?
C2
pyogenic: C1-4 (mainly C3)
Neisserial:
C5-9
Which complement def presents with hereditary angioedema?
C1 esterase inhibitor deficiency
- swelling is similar to hives but it is under the skin.
Which complement def. presents with complement mediated lysis of RBCs and paroxysmal nocturnal hemoglobinuria?
DAF (GPI- anchored enzyme) deficiency
Decay accelerating factor helps prevent complement
activation on self cells
Most common serious primary immuno defect in adults
Common variable immunodeficiency (CVID)
Low fxn in these tests are indicative of what type of immunodeficiency - Phagocytic, cellular, or humoral immunity?
- NBT
- Skin tests
- Anti RBC
- Vaccination
- NBT: phagocytic
- sufficient phagocyte # but decreased fxn - Skin tests: Cellular
- Anti RBC: humoral
- Vaccination: Humoral
Low # in these tests are indicative of what type of immunodeficiency - Phagocytic, cellular, or humoral immunity?
1. Lymphs
- Lymphs less than 1500
- cellular immunodef.
