LEC 06 Primary Immunodeficiency Approach to Patient with Recurrent Infections Flashcards by The Amazing Penisman

What are the 3 main functions of the immune system

Defense, Homeostasis, Surveillance

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The immunopathological state from failure of appropriate recognition.

Autoimmunity

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The immunopathological state of overactive immune responses.

Hypersensitivity

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The immunopathological state of failure to produce an adequate immune response.

Immunodeficiency

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The complex sequence of events triggered by the introduction of a stimulus and usually culminates in the elimination of the provoking agent.

Immune Response

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[Immune Response] The 1st Line of Defense is:

a. non-specific
b. innate
c. natural
d. present in all healthy individuals
e. AOTA

e. AOTA

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True or False: Cytokines and chemokines are components of the 1st Line of Defense

True.

Other soluble factors:

 Antimicrobal enzymes (lysozyme)
 Complement
 Cytokines/Chemokines
 Acute Phase Reactants (ESR, 
CRP)*

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True or False: Cell-mediated immunity is under the 2nd Line of Defense

True.

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1st or 2nd Line of Defense?

Consists of anatomic barriers such as the skin, mucus membrane and cilia.

1st.

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1st or 2nd Line of Defense?

Acquired immune responses.

2nd.

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1st or 2nd Line of Defense?

Resistance improved by repeated infection.

2nd.

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1st or 2nd Line of Defense?

No prior exposure needed for it to be functional.

1st.

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The 4 Major Host Defense Systems:

Antibody-mediated/B-cell immunity (adaptive)
Cell-mediated/T-cell immunity (adaptive)
Phagocytic cells (innate)
Complement system (innate)

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The pathogenesis of Immunodeficiency involves which of the following?

a. genetic defects
b. chromosomal abnormalities
c. drugs or toxins
d. nutritional and metabolic imbalances
e. AOTA
f. NOTA

e. AOTA

Immunodeficiency can result from:

Genetic Defects
Chromosomal abnormalities
Drugs/toxins
Nutritional and metabolic imbalances
Infection

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Most common genetic defect that can lead to Immunodeficiency:

a. Single-gene expressed in multiple tissues (ataxia-telangectasia)

b. Single-gene defects specific to immune system (BTK defects
in XLA)

c. Multifactorial disorders with genetic susceptibility (CVID)
d. NOTA

b. Single-gene defects specific to immune system (BTK defects in XLA)

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The deletion of gene 22q11 can lead to this chromosomal abnormality which can cause immunodeficiency.

DiGeorge anomaly

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Which of the following can lead to TRANSIENT immunodeficiency?

a. measles
b. HIV
c. congenital rubella
d. chancroid

a. measles

Transient immunodeficiency (measles, varicella)
Permanent immunodeficiency - (HIV, congenital rubella)

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What are the hallmarks of immunodeficiency?

Chronic infections
Recurrent infections
Presence of unusual infections
Presence of unusual sites of infections
Infections with common childhood pathogens but of unusual severity
Failure to thrive

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The 10 Warning Signs of Immunodeficiency (Pediatrics)

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1) 8 (Adult:2) or more new ear
infections within 1 year

2) 2 or more serious sinus
infections within 1 year (A: in the absence of allergies)

3) 2 or more months on
antibiotics with little effect (A: recurrent viral infections with colds, herpes, warts & condyloma)

4) 2 or more (A:1) pneumonias
within 1 year

5) Failure to thrive (failure of
an infant to gain weight or
grow normally (A: weight loss due to chronic diarrhea)

6) Recurrent deep skin or organ abscesses (A: same)

7) Persistent oral thrush/other areas in the skin after 1 years
old (A: same)
8) Need for IV antibiotics to clear infections (A: same)

9) 2 or more deep-seared
infections (meningitis,
osteomyelitis, cellulitis,
sepsis) (A: Infection with normally harmless mycobacterium)

10) Family history or primary immunodeficiency (A: same)

Identify the arm of immune system that is affected [B-cell, T-cell/combined, Phagocytic, or Complement]:

Recurrent viral infection

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T-cell (+++) and B-cell (+)

Identify the arm of immune system that is affected [B-cell, T-cell/combined, Phagocytic, or Complement]:

Associated only with recurrent bacterial infections.

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Phagocyte deficiency

Identify the arm of immune system that is affected [B-cell, T-cell/combined, Phagocytic, or Complement]:

Recurrent bacterial infection.

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ALL

Identify the arm of immune system that is affected [B-cell, T-cell/combined, Phagocytic, or Complement]:

Fungal infections.

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T-cell (+++) and Complement deficiency (++)

Most important red flag for immunodeficiency

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failure to thrive

Stage 1 of Immunologic Testing for suspected Immunodeficiency:

1. History, physical exam, height and weight 2. CBC and differential (look for absolute neutrophil and lymphocyte count) 3. Quantitative immunoglobulin levels (IgG, IgM, IgA – age related values) 4. CXR (check for thymic shadow) * basically parang general check-up

Stage 2 of Immunologic Testing for suspected immunodeficiency:

1. Specific antibody responses (tetanus, diphtheria, Hep B) 2. Responses to pneumococcal vaccine 3. IgG subclass analysis

Stage 3 of Immunologic Testing for suspected immunodeficiency:

1. Candida, PPD, and tetanus skin test 2. T- and B-cell enumeration 3. Neutrophil oxidation burst (if indicated)

Stage 4 of Immunologic Testing for suspected immunodeficiency:

1. Complement screening (CH 50, C3, C4) 2. Family/genetic studies 3. Enzyme measurements 4. Phagocytic measurements 5. NK cytotoxicity studies 6. Cytokine receptor studies

Majority of primary immunodeficiency cases are due to deficiencies in: a. Phagocyte b. T-cells c. B-cells d. Complement

c. B-cells (>50%)

Clinical presentation for patients with B-cell immune deficiency disorders:

ADRE - Age>6 months - Diarrhea and malabsorption - Recurrent sinopulmonary infections (usually mucous membranes) - Encapsulated high grade bacteria

Condition of pregnancy where fetus has the lowest levels of IgG

Hypogammaglobulinemia

Adult levels of IgM are achieved by ____ ; IgG at ____

IgM: 1 year; IgG: 5 years

The first immunoglobulin to develop in a newborn:

IgM

Site an instance where IgM levels are unusually high in a newborn.

Intrauterine infection by TORCH

Initial tests for B-lymphocyte function:

PAQI (nitial) - Protein electrophoresis - Antibody responses to immunization - Quantitative Ig (G,A,M) - Isohemaglutinins

Advanced tests for T-lymphocyte function:

IBA (dvanced) - IgG subclass quantitation - B-lymphocyte quantitation (CD19 or CD20) - Antibody responses to pneumococcal polysaccharides

Clinical presentation of T-cell/ Combined immune deficiency disorders:

FOPRR - Failure to thrive - Opportunistic infections - Presentation in first 6 months - Recurrent diarrhea - Recurrent candidiasis

Initial tests for T-lymphocyte function:

- Total lymphocyte count (lymphopenia <1500 cells) - Delayed-type hypersensitivity skin tests (PPD, Candida, Tetanus, Mumps, Trichophyton) - CXR (infants)

Advanced tests for T-lymphocyte function:

1. T-lymphocyte quantitation 1. a. Total T-cells (CD3) 1. b. T-helper cells (CD4) 1. c T-suppressor/ cytotoxic cells (CD8) 2. Proliferative responses to mitogens, antigens, allogeneic cells

True or False: Stem cell transplantation is indicated for Primary Immunodeficiency.

TRUE.

Correct initial dose for IVIg for Primary immunodeficiency:

400-600 mg/kg/dose every 3-4 weels

Clinical presentation for Phagocytic disorders:

- Recurrent skin infections (abscesses), poor wound healing - Mucosal infections and periodontal disease - Sepsis - Delayed umbilical cord separation (in Leukocyte Adhesion Deficiency) (usually detaches by 1-2 weeks, max at 1 month) - Organisms: catalase positive bacteria (S. aureus, Pseudomona) and fungi * Catalase neutralizes ROS that kill most bacteria.

Tests for phagocytic function:

INITIAL (WiNneR)  WBC count/morphology  Nitrotube Terazolium Reduction Test (NBT)  Rebuck skin window ``` ADVANCED (PC BaN)  Phagocytic assay  Chemotaxis assay  Bactericidal assay  Neutrophil oxidative burst ```

Clinical Presentation of Complement Component Deficiency:

- Recurrent pyogenic infections and connective tissue diseases (especially C2 and C4) - Recurrent Neisseria species infection (due to deficiency of component C5 to C9) - C1 esterase inhibitor deficiency associated with hereditary angioedema

Tests for Complement Function:

INITIAL  CH50 (hemolytic component)  C3 and C4 antigen levels ADVANCED  Serum opsonic and chemotactic assays  Individual component assays

True or False: Secondary Immune Deficiency is more common than Primary Immune deficiency.

TRUE. Primary: inborn Secondary: acquired

LEC 06 Primary Immunodeficiency Approach to Patient with Recurrent Infections Flashcards

(46 cards)