Module 2.4 Immune disorders Flashcards

(66 cards)

1
Q

Primary Immune disorders

A

Congenital or inherited

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2
Q

Why are primary disorders increasing in number over time

A

increased awareness and diagnosis. NOT increased incidence.

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3
Q

Humoral Immunity

A

B-cells, antibody immunity

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4
Q

Cell Mediated Immunity

A

T-Cell

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5
Q

4 Major Categories of Immune system

A

Humoral
Cell-Mediated
Compliment System
Phagocytosis (bridge between innate and acquired)

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6
Q

B-cell immunodeficiency is associated with

A
Enteroviruses
Streptococcus
H. Influenzae
Giardia lamblia (protozoa)
NO FUNGALS
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7
Q

T-Cell Immunodeficiency is associated with

A

Herpes
Salmonella
Mycobacteria
Candida Albicans and many other fungal infections

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8
Q

Combined T-cell and B-cell immunodeficiency is associated with

A

All viruses
Neisseria Meningitis
Toxoplasmosis gondii

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9
Q

Allografts

A

from other people

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10
Q

Autografts

A

From self

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11
Q

Transient Hypogammaglobulinemia of infancy

A

transient deficiency in some infants. Causes recurrent pyrogenic illnesses

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12
Q

B-cell immunodeficiency is usually not associated viruses. Why? What’s the exception?

A

B-cells don’t engage with intracellular pathogens.

Exception: enteroviruses

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13
Q

What causes x-linked agammaglobulinemia

A

Pre-B Cells fail to migrate to lymphoid tissue resulting in no immunoglobulin production at all

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14
Q

DiGeorge Syndrome

A

Primary T-Cell Deficiency
Congenital heart disease
Hypocalcemia
Complete or partial failure of Thymus and parathyroid development.

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15
Q

Most common primary immunodeficiency

A

Primary Humoral (B-cell) deficiency. (70%)

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16
Q

Secondary Cell Mediated immunodeficiencies are caused by

A

Acute viral syndromes

some cancers

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17
Q

Severe Combined Immunodeficiency (SCIDs)

A

X-linked inheritance

Combined T cell and B cell deficiency

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18
Q

Ataxia–telangiectasia

A

Autosomal Recessive Inheritance

Combined T cell and B cell deficiency

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19
Q

Ataxia–telangiectasia S/S

A
Occulocutaneous telangiectasis (big red vessels in eyes).
Ataxia (kid cant walk when it's time to learn).
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20
Q

Ataxia-telangiectasis immune effects

A

T-Cell function unchanged but decreased in number

Deficiency in IgA IgE and IgG

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21
Q

Wiskott-Aldrich Syndrome

A

X- Linked recessive (affects boys primarily)

Combined T cell and B cell deficiency

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22
Q

Wiskott-Aldrich Syndrome S/S

A

Thrombocytopenia
Recurrent Infections
Eczema

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23
Q

Wiskott-Aldrich Syndrome Prognosis

A

life expectancy 15 years

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24
Q

Wiskott-Aldrich Syndrome immune effects

A

Low IgM
High IgA and IgE
Limited T-cell function which improves with time

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25
Wiskott-Aldrich Syndrome treatment
Stem Cell or Bone Marrow transplant
26
How are most primary compliment system disorders acquired
autosomal recessive
27
Hereditary angioneurotic edema is associated with
Primary compliment system disorders.
28
Hereditary angioneurotic edema is caused by
Swollen mucous membranes (airway, lips, deep localized tissue swelling)
29
What causes Secondary compliment system disorders
Rapid activation -> depletion or reduced synthesis of compliment components
30
Dysfunction of the phagocytic system would make one particularly susceptible to:
FUNGAL INFECTIONS! Candida species Filamentous fungi May also result in chronic granulomatous disease (CGD)
31
Hypersensitivity types and their associated systems
Type I - IgE mediated Type II - Antibody mediated Type III - Complement mediated Type IV - T-Cell Mediated
32
Type I hypersensitivity
Allergies! Causes release of histamines from MAST cells
33
Management of Type I
Avoid offending agent Nasal steroids Cromolyn Sodium (Mast cell stabilizer) Desensitization (allergy shots)
34
What is the basic principle behind desensitization
Increase IgG antibodies to the antigen so that IgG binds rather than IgE which is what causes the reaction.
35
Primary/initial phase of Type 1 response
IgE stimulates Mast cells, basophils and eosinophils. Vasodilation Vascular Leakage (swelling) Smooth muscle contraction
36
Secondary/late phase of Type 1 response
More intense infiltration of tissue with eosinophils | Endothelial cell damage
37
What causes the secondary/late phase response of type 1 hypersensitivities
T-cell recruitment of eosinophils + Degranulation of mast cells causing release of membrane phospholipids (arachidonic acid, prostaglandins, leukotriens).
38
What determines the severity of type 1 reactions
Degree of Sensitization (# of IgE receptors on mast cells or number of mast cells). NOT the quantity of exposure.
39
Grades of Type reactions
I: Cutaneous and Muscle Erythema, Urticaria. II: Multisystem S/S Hypotens. Tachycardia, dyspnea, N/V, Diarrhea III: Bronchospasm, dysrhythmias IV. Cardiac Arrest
40
ABC priority
BAC now
41
Type II (cytotoxic) reactions are mediated by
IgG and IgM
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Most common Type II reaction example
Blood transfusion reactions and Hemolytic disease of the newborn
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Which antibody causes hemolytic disease of the newborn
IgG - only one that can cross placenta
44
SEE PAGE 319 IN BOOK FOR TYPE 2 REACTION TABLE
SEE PAGE 319 IN BOOK FOR TYPE 2 REACTION TABLE
45
Serum Sickness
Systemic Type 3 (immune complex) reaction | Takes 1-2 weeks to manifest
46
Arthus Reaction
Localized Type 3 (immune complex) reaction
47
What causes type 3 reaction
Antibody-Antigen complex forms, binds to cell membrane causing compliment system activation which attracts inflammatory cells
48
Type IV hypersensitivity
T-cell mediated. | Can be Direct cell-mediated or Delayed Type
49
Delayed Type IV reaction
CD4 T cells release cell damaging cytokines | Contact dermatitis, farmer's lung,
50
Direct Cell Mediated Type IV reaction
CD8 T Cells kill antigen bearing target cells
51
Latex reactions are what type of reactions
Can be type I or IV | Symptoms determine the type
52
Transplanted tissue categories and definitions
Allogeneic - Similar HLA types between donor and recipient (may or may not be related) Syngeneic - Identical twins Autologous - Same person
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Central tolerance
Elimination of self reactive T-cells (in thymus) and B-cells (in bone marrow)
54
Peripheral tolerance
Deletion of auto-reactive cells that escaped central tolerance elimination
55
Hyperacute Transplant Rejection
occurs almost immediately. | Type II reaction, arthus reaction.
56
Acute Transplant Rejection
Occurs in first few months | T-Lymphocytes respond to antigens in graft tissue.
57
Chronic Host-Versus-Graft Rejection
Occurs over years | Fibrosis of blood vessels in transplanted organ. unknown mechanism
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Graft Versus Host Disease
Grafted organ attacks host body. takes 10-14 days
59
GVHD requirements
must be an organ with functional immune component Host immune system must be compromised to the point of being unable to destroy the transplanted organ.
60
GVHD Clinical manifestations
Palm and feet rashes
61
Systemic Lupus Erythematosus
Autoimmune disease Butterfly rash Causes systemic issues with most organs/systems
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Autoimmune Hemolytic Anemia
Antibodies attack host blood cells
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Pemphigus Vulgaris
Blistering of skin and Epithelial linings.
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Hashimoto Thyroid
first ever autoimmune disease discovered Causes hypothyroid
65
Diagnostic criteria for autoimmune disorders
Evidence of autoimmune reaction Determination that it's not secondary to another condition Lack of other identified causes Lab tests
66
Lab tests to demonstrate autoimmune reactions
Indirect Fluorescent Antibody Assay (IFA) Particle agglutination assay Enzyme Linked Immunosorbent assay (ELISA)