Hyper- & Hypo Immune Disorders Flashcards

1
Q

Hyper- & Hypo- Immune Disorders

A

Inadequate innate immunity
- Neutropenia, abnormal phagocytosis, complement system deficiency, hypersplenism
Excessive innate immunity
- Neutrophilia, monocytosis, asthma
Misdirected innate immunity
- Angioedema
Inadequate adaptive immunity
- T lymphocytes deficiency (DiGeorge Syndrome), SCIDs
Excessive adaptive immunity
- Allergic reactions, anaphylaxis, drug allergies
Misdirected adaptive immunity
- Hypersensitivity to self-antigens, SLE, rheumatoid arthritis, hepatitis
Anesthesia & immunocompetence
- Graft vs. host disease
- Tumor lysis syndrome

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2
Q

Innate Immunity

A

NON-SPECIFIC response that targets common pathogens
No prior exposure required to elicit response
Passed on to each generation
Epithelial & mucus membranes, complement factors, neutrophils, macrophages, & monocytes
RAPID response
Mediated via cells & plasma proteins that are always present
Principle cells = myeloid cells (macrophages, neutrophils, dendritic cells)
NOT pathogen specific

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3
Q

Adaptive Immunity

A

Developed individually
Delayed response, develops memory & specific towards antigen, B & T lymphocytes
Powerful, normally silent but active & adapt to antigens
Specialized, unique specificity
Receptors created by rearrangement antigen-receptor genes that occur during lymphocyte maturation
Principle cells = T & B lymphocytes

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4
Q

Humoral Mediated Immunity

A

Mediated by antibodies produced by B cells

Antibodies neutralize microbes, opsonize them for phagocytosis, & activate the complement system

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5
Q

Cell Mediated Immunity

A

T cells activated by protein antigens from antigen presenting cells (APCs)
Requires repeat antigen stimulation to perform their functions
CD4+ helper T cells secrete cytokines to activate macrophages, helps B cells make antibodies, & stimulate inflammation
CD8+ helper T cells kill infected & transformed cells

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6
Q

Adaptive Immune Dysfunction

A
Defects in antibody production or T lymphocytes
Combines immune system defects (SCIDs)
Allergic reactions
Anaphylaxis
Autoimmune disorders
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7
Q

-penia

A

Lack of, poverty, deficiency

Neutropenia = lacking neutrophils

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8
Q

-philia

A

Affinity, attraction, fondness

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9
Q

Allergy

A

Reactions against normally harmless environmental signs

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10
Q

Autoimmune

A

Reactions against self-antigens

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11
Q

Hypersensitivity

A

Excessive immunologic reactions to microbes or environmental agents dominated by inflammation

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12
Q

Atopy

A

Propensity or genetic tendency to develop allergic reactions

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13
Q

Antibody (Ab)

A

Immunoglobulin (Ig) large Y-shaped protein used by the immune system to identify & neutralize foreign objects such as pathogenic bacteria & viruses

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14
Q

Neutrophils

A

Formed by stem cells in the bone marrow
Make up 40-70% all WBCs in humans
Phagocytes found in the bloodstream
FIRST RESPONDERS to inflammation - especially bacteria
Predominant cells in pus (create yellow/white-ish color)

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15
Q

Neutropenia

A

<1,500/mm^3
Types include neonatal sepsis, Kostmann syndrome, acquired defects, autoimmune, infection
Treatments include medication cessation, granulocyte colony-stimulating factor, & bone marrow transplants
ASEPSIS important

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16
Q

Spleen

A

Lymphatic system
Large lymph node - primary blood filter
Primary RBC creation site fetal up to 5mos
Function: 250mL blood reservoir, removes old RBCs, recycles iron, metabolizes hemoglobin, lymphocyte storage, clears platelets
Globin → amino acids
Heme → bilirubin (removed via liver)
Synthesizes antibodies

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17
Q

Asplenia

A

Absence normal spleen function
Type immuno-dysfunction
Increased sepsis risk 350x d/t spleen unable to clear bacteria from the blood

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18
Q

Hyposplenism

A

Reduced spleen function

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19
Q

Sickle Cell Anemia

A

Auto-infarction w/in spleen results in vaso-occlusive disease

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20
Q

Leukocytosis

A

WBC count above normal range
Normal reaction - inflammatory response
Other causes include tumor, leukemias, pregnancy, convulsions & medications

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21
Q

Left Upper Shift

A

↑ratio immature to mature neutrophils

Bone marrow trying to make more

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22
Q

Right Shift

A

↓ratio immature to mature neutrophils

Shows bone marrow suppression (radiation sickness)

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23
Q

Leukemia

A

Acute - immature WBCs present in the peripheral blood

Chronic - mature, non-functioning WBCs in peripheral blood

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24
Q

Eosinophilic Esophagitis

A

Chronic immune system disease where type WBC (eosinophil) build-up in esophagus lining
Build-up reaction to foods, allergens, or acid reflux → inflame or injure the esophageal tissue
Damaged tissue → difficulty swallowing or cause food to get stuck

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Neutrophilia
Granulocytes ↑2-3x Neutrophilia >7,000/mm^3 - Pancreatitis, pyelonephritis, peritonitis, pneumonia Leukostasis >100,000/mm^3 - Thick blood flow & WBC clumping → TIAs & strokes Myeloproliferative disorder or hematologic malignancy >50,000/mm^3
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Asthma
Exaggerated bronchoconstriction response to stimuli
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EXTRINSIC Asthma
IgE production | Allergens
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INTRINSIC Asthma
Triggers are unrelated to the immune system | Examples: ETT placement, cold, exercise, stress, inhaled irritants
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Angioedema
Hereditary or acquired Subcutaneous & submucosal edema formation Often involves face, extremities, & GI tract
30
Bradykinin-Mediated Angioedema
Autosomal dominant deficiency/dysfunction C1 esterase inhibitor ACEi drug-induced angioedema d/t ↑bradykinin Acquired - lymphoproliferative disorders acquire C1 esterase inhibitor deficiency 2° antibody production CATECHOLAMINE & ANTIHISTAMINES ARE NOT EFFECTIVE IN ACUTE EPISODES
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Acute Angioedema Treatment
Androgens - prophylactic therapy Antifibrinolytic therapy inhibiting plasmin activation C1 inhibitor concentrate Synthetic bradykinin receptor antagonist Recombinant plasma kallikrein inhibitor - blocks kininogen → bradykinin conversion FFP replaces the deficient enzyme
32
DiGeorge Syndrome
Thymic, thyroid, & parathyroid hypoplasia Cause: 22q11.2 gene deletion ↓T cells (B cells are normal) Cardiac malformations & facial dysmorphisms - Truncus arteriosus & TOF - Cleft palate Immunocompromise degree correlates w/ amount thymus tissue present Complete absence = severe combined immunodeficiency syndrome Treatment: T-cell infusion or thymus transplant Hypoparathyroidism - Ca2+ supplementation Strict asepsis d/t infection risk
33
Severe Combined Immunodeficiency
Genetic mutations that affect T, B, & NK cell function/maturation X-linked form 1/58,000 births Appear healthy at birth but highly susceptible to severe infections All newborns screened SCIDs Gene mutations that encode for interleukin receptors Treatment: bone marrow or stem-cell transplant, gene therapy, or enzyme replacement
34
Allergic Reactions
Immune-mediated "Overreactions" 4 types: Type I - IgE (anaphylaxis) Type II - IgG/IgM (myasthenia gravis, Grave's disease) Type III - immune complex (SLE) Type IV - T lymphocytes (rheumatoid arthritis, multiple sclerosis)
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Type I
IgE Histamine release & other mediators from mast cells Vascular dilation, edema, smooth muscle contraction, mucus production, tissue injury, inflammation Immediate hypersensitivity Anaphylaxis, allergies, bronchial asthma
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Type II
IgG/IgM production binds to antigen on target cell or tissue → phagocytosis or lysis target cell via activated complement Leukocytes recruitment Possibly delayed Autoimmune hemolytic anemia
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Type III
Immune complex formation (antigen-antibody complexes) → complement activation Recruit leukocytes Inflammation, necrotizing vasculitis (fibrinoid necrosis) Delayed SLE, glomerulonephritis, serum sickness
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Type IV
Activated T lymphocytes Release cytokines, inflammation, & macrophage activation T cell-mediated cytotoxicity Perivascular Delayed Chronic dermatitis, multiple sclerosis, type 1 diabetes, tuberculosis
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Anaphylaxis
Life-threatening Cardiovascular collapse (tachycardia, hypovolemia) Interstitial edema, urticaria (cutaneous rash) Bronchospasm, laryngeal edema Immune mediated IgE 60% Non-immune mediated "anaphylactoid" IgG or IgM Less common Direct histamine release from mast or basophils
40
Histamine
Vasoactive amine Stored in mast cells Release upon mast cell degranulation Causes → vasodilation, ↑vascular permeability, & smooth muscle contraction
41
Prostaglandins
``` Lipid mediator Prostaglandin D2 (PGD2) most abundant mediator generated by cyclooxygenase pathway in mast cells → intense bronchospasm ```
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Leukotrienes
Lipid mediator | Most potent vasoactive & spasmogenic agents known
43
Cytokines
Tumor necrosis factor & chemokines | Recruit, activate, & amplify leukocytes
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Perioperative Anaphylaxis Management
``` Remove triggering agent Reverse/treat hypotension & hypoxemia Replace intravascular fluid Inhibit further degranulation Inhibit release vasoactive mediators Treat inflammation Relieve bronchospasm ```
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Antihistamines
Histamine 1 antagonist Diphenhydramine competes w/ histamine Histamine 2 antagonist Ranitidine ↓pruritis & bronchospasm Not as effective to treat anaphylaxis once vasoactive mediators have been released
46
Epinephrine
1-10mcg/kg IV bolus Repeat every 1-2minutes as needed ↑intracellular cAMP, restores membrane permeability, & ↓release vasoactive mediators β agonists relax bronchial smooth muscle UNRESPONSIVE TO EPI → VASOPRESSIN, GLUCAGON, OR NOREPINEPHRINE
47
β2 Agonists
Albuterol delivered via MDI or nebulizer useful to treat bronchospasm
48
Corticosteroids
Several hours to take effect Potentially enhance β agonist effects Inhibit arachidonic acid release (leukotriene & prostaglandin production)
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Intolerance
Inability to tolerate medication adverse effects | Example: muscle pain & statins
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Idiosyncratic Reactions
Drug reactions not r/t known pharmacological drug properties | Example: antiepileptic drugs & dyskinesias
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Toxicity
Dose-dependent | Too much drug present in patient system at one time
52
Perioperative Anaphylaxis Causes | MUSCLE RELAXANTS
Rocuronium & Succinylcholine | Atracurium histamine release = non-immune mediated
53
Perioperative Anaphylaxis Causes | ANTIBIOTICS
PCN & cross-sensitivity w/ cephalosporins (β-lactam ring) Sulfonamide - second most common (Stevens-Johnson syndrome) Vancomycin - non IgE mediated (direct histamine release r/t drug infusion rate)
54
Perioperative Anaphylaxis Causes | LATEX
Delayed onset >30min after exposure | Spina bifida, multiple previous operations, fruit allergy, & healthcare workers
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Perioperative Anaphylaxis Causes | PROPOFOL
``` Contains lecithin (derived from egg yolk) & soybean oil as emulsifying agents Preservatives = EDTA or sodium metabisulfite/benzoate 2-isoproyl group ```
56
Perioperative Anaphylaxis Causes | ASA & NSAIDs
Rhinorrhea, bronchospasm, & angioedema High risk patients = asthma, hyperplastic sinusitis, & nasal polyps NON IgE mediated Cyclooxygenase-1 inhibition promotes leukotriene synthesis → release mediators from basophils & mast cells
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Perioperative Anaphylaxis Causes | RADIOCONTRAST MEDIA
0.1-3% More common w/ ionic, high-osmolar contrast agents ↑iodine ↑risk adverse reaction Non-immune mediated pretreat w/ corticosteroid & histamine antagonists
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Perioperative Anaphylaxis Causes | OTHER
Midazolam, Etomidate, Ketamine, Heparin, Insulin Opioids - Morphine, Codeine, & Meperidine directly release histamine Local anesthetics <1% Ester > Amide Halothane induced hepatitis Dyes - ICG or methylene blue Chlorhexidine Synthetic volume expanders - contain dextrans, gelatins, albumin, starch → both immune & non-immune reactions Blood products
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Rejection
Histocompatibility determines if tissue graft will be accepted (compatible) by the receiving individual Major histocompatibility complex (MHC) are polymorphic genes that differ among individuals - Function to recognize T cells Graft donor expresses MHC molecules differ from those in the recipient host - Graft recognized as foreign by recipient T cells - Recipient CD4+ & CD8+ T cells specific for graft antigens are activated, migrate back into transplants, & cause its rejection
60
Graft Rejection Treatment
IMMUNOSUPPRESSION needed to prolong graft survival - Corticosteroids, anti T-cell antibodies, T-cell function inhibition drugs Immunosuppression → risk opportunistic fungal & viral infections - Reactivation latex viruses (cytomegalovirus) ↑cancer risk in immunocompromised patients
61
Graft vs. Host Disease
Syndrome commonly associated w/ bone marrow & stem cell transplants Donor WBCs remain w/ the donated tissue (graft) recognize the recipient (host) as foreign NOT the same as transplant rejection Donor immune system rejects the recipient body Treatment: T-cell suppression - Steroids & calcineurin inhibitors (cyclosporin & tacrolimus) - Suppresses pro-inflammatory cytokine synthesis
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Tumor Lysis Syndrome
Rare but potentially lethal Massive lysis tumor cells results in intracellular substances release into the bloodstream - Potassium, phosphate, uric acid Causes: steroids & after chemoembolization or radiofrequency ablation treatments