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Immuno Block Mid Term 2 > Primary Immune Deficiencies > Flashcards

Primary Immune Deficiencies Flashcards

1
Q

most prominent type of primary immune deficiency

A

a majority of the cases are due to humoral defects with immunoglobulins and B cells

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2
Q

common signs/symptoms of humoral deficiency

A

pyogenic infections with recurrent otitis media, sinusitis, pneumonia

frequent viral infections, chronic diarrhea

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3
Q

too many infections in kids?

A

4 courses of antibiotics in a year

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4
Q

too many infections in adults?

A

2 courses of antibiotics in a year

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5
Q

too many ear infections after age 4?

A

4 in a year, less because physiology changing

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6
Q

Agammaglobulinemia cause

A

defect in B cell development, germinal center formation is defective

often see under development of lymphoid tissue like nodes, spleen, tonsils

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7
Q

X linked Agammaglobulinemia etiology and name

A

85% of agammglobulinemias, called Brutons agammaglobulinemia

due to problem with B cell tyrosine kinase

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8
Q

BTK receptor in Brutons Agammaglobulinemia

A

part of the pre B cell receptor that leads to a phosphorylation and pathway to stimulate B cell maturation

one protein in a complex so other proteins can have problems too in the same complex

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9
Q

XLA cellular signs

A

IgG less than 100 mg/dl
B cells less than 2% of total lymphocytes
Normal T cell function and number

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10
Q

Hyper IgM syndrome

A 
problems with class switching of antibodies
have regular number of B cells but elevated levels of IgM and low IgE/A/G
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11
Q

IgA deficiency information

A

IgA less than 5-7mg/dl
most common primary immunodeficiency
Usually asymptomatic

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12
Q

Common Variable Immunodeficiency (CVID) cell signs

A

most common symptomatic primary immunodeficiency

low IgG/A/M, reduced specific antibody responses to previous infections or vaccines

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13
Q

Cause of Common Variable Immunofdeficiency

A

usually a gene involved in the development or maturation of B cells

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14
Q

Common Variable Immunodeficiency symptoms

A

recurrent infections, mainly sinusitis, and pneumonia

life threatening infections

poor response to vaccines

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15
Q

Risks associated with Common Variable Immunodeficiency

A

granulomatous diseases, autoimmune disorders, splenomegaly, and malignancies like lymphoma

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16
Q

Specific Antibody Deficiency cell signs

A

normal antibody levels, normal T and B cell number and function

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17
Q

Specific Antibody Deficiency symptoms

A

usually have recurrent sinopulmonary infections, impaired antibody response to encapsulated bacteria

most common deficiency is the specific antibody deficiency to pneumococcus from vaccine (polysaccharide)

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18
Q

Some Killer Have Pretty Nice Capsules (encapsulated mnemonic

A 
Step pneumo and pyogenes
Staph Aureus
Klebsiella
Haemophilus Influenzae
Pseudomonas aeruginosa
Neisseria meningitidis 
Cryptococcus neoformas
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19
Q

Transient hypogammaglobulinemia in infancy symptoms

A

recurrent sinopulmonary infections

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20
Q

Transient hypogammaglobulinemia in infancy cell signs

A

low IgG but normal specific antibodies due to slowed response

normal lymphocyte count and function

resolves by age 4, onset after IgG maternal lost

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21
Q

Tests to run if speculating humoral deficiency?

A 
CBC with differential
Age adjusted quantitative immunoglobulins
Specific antibody titers
Complement pathway CH50, AH50
Lymphocyte markers (CD19)
22
Q

common Specific antibody production tests

A

tetanus and diphtheria proteins and isohemagglutinins

23
Q

Prevnar vaccine for pneumococcal

A

conjugate vaccine given to infants that combines proteins from 13 common penuomoccocal strands

24
Q

Pneumovax polysaccharide vaccine for pneumococcal

A

combines 23 common polysaccharides from different pneumococcal strands

25
Q

common clinical infections withT cell dysfunction

A

infections with intracellular organisms like viruses, protozoa, mycobacterium, fungal, encapsulated bacteria

26
Q

Common clinical symptoms with T cell dysfunction

A

lack of growth, cant recall antigens, thrush, eczema, hair loss, eosinophilia, increased B cell malignancies

27
Q

Chronic Mucocutaneous Candidiasis symptoms

A

result in decreased cell mediated immunity against Candida species

chronic non invasive infections usually cause changes in skin, nails and mucosal membranes

28
Q

SCID age of onset

A

typically between 4-6 months of age

29
Q

SCID signs of disease

A

severe persistent infections, oral thrush, chronic diarrhea and lack of growth

30
Q

SCID cellular cause

A

super low numbers of T cells…sometimes like B or Nk cells too

will not respond to T cell mitogen test

31
Q

IL-2R gamma chain SCID cellular effects, mode of inheritance and defect

A

T cells low, B cells regular and NK cells low

X linked

defect in common gamma chain that makes interleukins

32
Q

Adenosine Deaminase Deficiency in SCID cellular effects, mode of inheritance and defect

A

T, B, and NK cells are low

AR

accumulation of toxic purine nucleosides…positive is you can give the enzyme to fix this

33
Q

RAG1/2 deficiency in SCID cellular effects, mode of inheritance and defect

A

T and B low, NK normal

AR

defective VDJ recombination

34
Q

TRECs definition

A

T cell receptor excision circles are made when the TCR complex is being produced

the TRECs are a sign of thymic function and T cell production…so if they are there it is unlikely to have SCID but if they are not there in a screening then you may have SCID

35
Q

TRECs role in screening for SCID

A

the TRECs are a sign of thymic function and T cell production…so if they are there it is unlikely to have SCID but if they are not there in a screening then you may have SCID

36
Q

Omenn Syndrome symptoms and cellular problems

A

commonly issues with RAG genes, is a subset of SCID called Leaky SCID

Low to normal number of T cells

onset about 3 months (earlier than SCID)
Symptoms include lymphadenopathy, heptosplenomegaly, diarrhea, lack of growth, elevated IgE and eosinophilia

37
Q

Wiskott aldrich syndrome and symptoms

A

involved with actin polymerization andaffects various lymphocyte functions

Eczema, small platelets, low MPV, low IgG and IgM

Regular IgA and IgE

high risk of autoimmune and malignancy

38
Q

T cell immunodeficiency Tests

A 
CBC with differential
mitogen testing
Antigen reactivity testing
DTH to candida
nucleic acid enzyme assays
39
Q

gene linkage of CGD

A

X linked and AR for phox genes

40
Q

Hyper IgE syndrome signs and symptoms

A

recurrent staph abscesses is most common

sinopulmonary infections and sever eczema

retained primary teeth, recurrent candida, recurrent bone fractures, high IgE, peripheral eosinophilia

41
Q

Hyper IgE cellular causes

A

IgE elevation is just a marker for disease

true cause is from problem with neutrophils and T cells

42
Q

Primary Immunodeficiencies with Onset of birth to 3 months

A

phagocytic cell defects, complement defects, Digeorges

43
Q

Primary Immunodeficiencies with Onset of 3-6 months

A

SCID

44
Q

Primary Immunodeficiencies with Onset of 6 to 18 months

A

X linked agammaglobulinemia and transient hypogammaglobulinemia

45
Q

Primary Immunodeficiencies with Onset of 18 months to adult

A

common variable immunodeficiency and complement defects

46
Q

treatments for humoral antibody deficiency

A

prophylactic antibiotics

IVIG and SQIgG

47
Q

treatments for combined immunodeficiency

A 
stem cell
IVIG
prophylactics
enzymes for PEG-ADA
gene therapy 
Thymic transplant in digeorges
48
Q

treatments for phagocytic cell defects

A

prophylactic antibiotics, avoid live viral vaccines (hyperIgE), gamma interferon in CGD, bone marrow

49
Q

kawasaki disease presentation

A

conjuctivitis, swollen lymph nodes, swollen hands/feet, rash, changes in oral mucosa

4/5 means kawasaki, IVIG and aspirin

can lead to vasculitis and increased risk of coronary artery disease

50
Q

should you give vaccine when on steroids?

A

maybe not because the steroids tone down CMI mainly and can cause the vaccine to not be as effective

Immuno Block Mid Term 2 (10 decks)

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