Immunopathologies/Immunology Flashcards

1
Q

Contact Dermatitis

A

Type IV, reaction to topic chemicals like henna, latex, nickel, hair dyes, tattoos

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2
Q

Rheumatic Heart Disease - Symptoms

A

Heart Murmur

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3
Q

Infections associated with T-cell Defieincy

A

Candida Albicans, Pneumocytsis Jirovecii (bactieral, virus, yeast, fungi)

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4
Q

Dressler’s Syndrome Treatment

A

usually self limited, stops when heart heals; or with autoimflammatory

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5
Q

CVID - mechanism

A

Normal Pre-B and B-cells, but B-cells are unable to make antibody intermittently; group of ~20 conditions

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6
Q

second graft reaction

A

45-10 days due to memory cells

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7
Q

Chronic Beryllium disease Mixed

A

4: T cells damage (HLA-DP1); CFR

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8
Q

Rheymatoid Factor

A

Type 3, pts serum to IgG coated microbeats. IgM anti-IgG will agglutinate.

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9
Q

Arthus Reactions

A

Type 3: if preexisitng ab (during booster) will form complex at injection site and causes fussiness and sore arm

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10
Q

What did David - Bubble Boy die from?

A

EBV infection - latent in everyone except we have functioning T-cells

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11
Q

CVID - name

A

Common Variable Immunodeficiency

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12
Q

Diagnosis of Asthma

A

Spirometry - measure air flow. Forcibly exhaled form full lungs (1 sec) + same with broncodilater. If improvement = asthma

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13
Q

Release of sequestered antigen

A

method in Type II immunopathy; immune response is generated to release antigen from store

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14
Q

Mutation in Celicas

A

90% are HL-DQ2

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15
Q

Prevalence of SCID

A

50 in 100,000

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16
Q

Type II immunopathy - basic

A

IgG, IgM, IgA autoantibodies to specific Host Tissue

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17
Q

Viral illnesses with secondary immunodeficiency

A

mononucleosis, cytomegalovirus, AIDs

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18
Q

Why does a TB test work?

A

Intradermal injection of antigen (PPD) that measure Th1 activation. (Type IV)

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19
Q

DiGeorge Syndrome Mechanism

A

Large 45 deletion on Ch22 to cause absence of parathyroid gland due to ineffective stroma of pharyngeal cleft. Most due to repeated mutations or de-novo. Absent T-cells, normal B-cells

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20
Q

Prevalence of Bruton’s

A

0.4 in 100,000

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21
Q

Myasthenia Gravis - Treatment

A

Thymectomy, immunosupresssion, nestigmine - drug to increase ACh effectiveness

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22
Q

Rheumatic Heart Disease Pathophys

A

Type II; Cross reaction between Group A Strep M protein to Laminin protein on heart valves, followed by neutrophil mediated destruction

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23
Q

Hyperacute graft reaction

A

Rejection before healing, bloodless graft, common in human-animal transplants due to predisposing antibody

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24
Q

Paroxysal Cold Hemobloginuria

A

a type of AIHA that causes hemolysis after exposure to cold.

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25
Q

Rheumatoid arthritis

A

Type 3,, trapped IgM to IgG complexes; elemetns of type 2 and 4

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26
Q

Autosomal Recessive SCID

A

Adenosine Daminase Deficiency ADA

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27
Q

Prevalence of DiGeorge

A

30 in 100,000

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28
Q

BCG vaccine

A

Vaccine against TB

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29
Q

Autoimmunity to Interferon Gamma

A

appears to be immunodeficient; often diagnosed due to rapid onset of TB in previously healthy patient

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30
Q

secondary immunodeficiency

A

immunodeficiency due to immunosuppressant drugs, advanced cancer, measles, AIDS

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31
Q

Quantifero

A

uses PPD from human mixed with blood sample and measures IFN-gamma. Only active TB (from actual exposure) will elicit a reaction

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32
Q

Autoimmunity Thrombocytopenic Purpura - Pathophys

A

Platelets are opsonized by AutoAb for destruction in spleen, Type II

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33
Q

Hybrid

A

method in Type II immunopathy; foreign antigen couples to self so anti-self B-cell ingest and normal Tfh is activated by foreign antigen to help class switch

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34
Q

Poison Ivey

A

urushiol oil causes delayed hypersensitivity reacion (Type IV)

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35
Q

Type III choroid plexus

A

confsion/dementia

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36
Q

Sjoren’s Syndrome

A

Type IV, unkown mechanism that causes CTL action against exocrine glans and dry saliva and tears

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37
Q

Symtoms of CVID

A

problems with innate immunity; recurrent bacterial infections, increased risk of lymphoma, enteropathy, autoimmunity, mild immunodeficiency compared to others

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38
Q

Mutations in SCID

A

SCID-X1; ADA; V(D)J recombination defects

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39
Q

Celiacs

A

Ab to gut endomyosium. Transglutaminase 2 (TG2) crosslinks normally with glutin, but in celiac it crosslinks with what gladden (digestion resistant) and becomes an antigen via an illicit help mechanism (T-cell mediated inflammation to gliadin) cause villi blunting

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40
Q

Nucala

A

reduces asthma by reducing blood eosinopohils

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41
Q

Histamine

A

itch, blood vessel dialation, leakiness

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42
Q

Renal biopsy in type 3

A

lumpy and bumpy with fluorsecence.

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43
Q

Symptoms of Bruton’s Agammaglobinemia

A

bacterial infections (less susceptible to viral infections), pneumonia, chronic diarrhea, enterovirus infections (due to no IgA) —> poliovirus

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44
Q

Chronic Frustrated Response

A

Noramlly TGFbeta+IL10 promote Treg, but dysregulation and release of TGFBeta and IL6 decrease Treg and increase TH1,2, 17 leading to chronic inflammation.

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45
Q

Basic Mechanism of SCID

A

Low T and B cell due to block in development of Lymphoid System; most severe

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46
Q

Ataxia Telangiectasia mechanism

A

Both T and B cell deficiency and severe IgA depression due to defect in DNA repair

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47
Q

Diagnosis of Type 3

A

History, CH50, Immune complexes in blood (cryoglubulins), Rhematoid factor, Renal biopsy,

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48
Q

Juvenile diabetes mixed

A

Type 2: ab to islet 4: T cells damage by HLA-DR3 and DR4

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49
Q

Oral Allergy Syndrome

A

Type I; food/antigen penetrates MM in mouth to activate mast calls, tingling lips and toungue, swelling, itching. From foods altho are similar to pollens

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50
Q

Type III immunopathology

A

Ab-Antigen complexes of intermediate size get stuck in basement membrane and activate complement (C1q); C3a and C5a atttrached neutrophils to release inflammatory mediators

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51
Q

Immunotherapy mechanism

A

favors IgG rpoduction so IgG traps and clears allergen before loaded on IgE mast cells.

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52
Q

Treatment of ADA

A

Irradiated blood (RBC are risk of adenosine deaminase to kill lymphocytes) or purified ADA Stabilized with polyethylene glycol

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53
Q

Hashimoto’s thyroiditis mixed

A

2: ab to thyroid; 4; tyroid infiltrated with T-cells to cause damage. Hypothyroidism

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54
Q

HyperIgM Mechanism

A

CD40 (on B cell) or CD40L (ligand on Tcell) is defective, so Tfh can’t recognize B-cell to help class switch.

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55
Q

Treatment of B-Cell Defienct Disorders

A

Human Ig - IVIG and SCIG given monthly

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56
Q

Autoimmune encephalitis

A

using brain cells as antigen and presenting to immune cycle to activate T-cells so they are more primed to enter BBB

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57
Q

Memory T-cell

A

decrease threshold of antigen exposure to elicit immune reponse

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58
Q

SLE

A

Type 3, IgG to dsDNA (own DNA) that deposits in the kidney (glomerulonephritis)

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59
Q

Type III pleura

A

pleusiy, effusion

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60
Q

infections associated with B-Cell Deficiency

A

Extracellular, pyrogenic - pus producing bacteria - staph, H influenza, Strep

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61
Q

Type I Immunopathology

A

allergic reaction that induces IgE, IgE beings to FceR1on mast cells that are cross linked to allergens to induce release of histamine.

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62
Q

Mutation in Bruton

A

Bruton Tyrosine Kinase (btk) defect, a tyrosine Kinase expressed in pre and mature B-cells

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63
Q

Chronic Spontaneous Uritcaria

A

IgG antibody to FceR1 on mast cells to chronically release histamine

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64
Q

Allergic seasonal rhinitis

A

hay fever, funny nose, itchy eyes in response to pollen, cats/dogs. Type I

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65
Q

Innocent Bystander

A

method in Type II immunopathy; damage to normal tissue that is associated or infected with antigen (drug that binds to RBC, ab to drug)

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66
Q

Diagnosis of Type I

A

hisotry, skin testing, RAST (ardio-allergo sorbent tesst) of ImmunoCAP-FEIA (samples serum combined with allergen fixed to capsule and added to serum. Unbound proteins washed away, and bound IgE revealed - safe method)

67
Q

Most SCID happen due to this mutation…

A

SCID-X1, an X linked Recessive defect in Gamma Chain that forms receptors IL-2

68
Q

Treatment of Immunodefiencies

A

Isolation; Prophylactic antibodies (combination, change monthly); Human Ig, Transplant

69
Q

Laboratory findings with Bruton’s

A

Pre-B cells in marrow; but deficiency in Bcells and antibody in periphery (Ig

70
Q

Mechanisms of Type II immunopathy

A

1) neutralization: human protein inactivated by autoAb 2)Complement: auto activates complement to induce lysis, phagocytosis, lysosomal enzymes 3) Stimulatory Hypersensitivity: autoAb directed against cell surface receptors and behaves like agonist

71
Q

One shot serum sickness

A

type III one shot of rabbit or horse serum to treat ID and 10-14 days later fever, malaise, rash, itch, arthralgia, hives, lymphadenopathy.; can be due to drug coupling with host proteins and viral infections

72
Q

Para-Phenylaminediame

A

black henna that elicits a DTH

73
Q

Signs/Symptoms of Digeorge

A

CATCH-22” Calcium (convlusions- inability to regulate calcium), Appearance (wide eyes, low set ears, fish mouth), Thymus (immunological disease), Cleft (palate), Heart (heart vessel abnormalities, tetralogy of flow, ASD, VSD)

74
Q

Multiple Sclerosis - pathophys

A

Type IV; T-cells reactive to Myelin Basic Protein. Brain is antigenic but NOT immunogenic

75
Q

Dressler’s Syndrome - Symptoms

A

Persistent cardiac pain, fever, malaise, pericardial effusion after heart attack or heart surgery

76
Q

Periodontal disease treatment

A

immunsuppresion, someontes ab to IL6 receptor

77
Q

Graves’ Disease

A

Stimulatory Autoimmunity, IgG resembles TSH to stimulate thyroid and cause hyperthyroidism; Type II

78
Q

Type III kidney

A

glomerulonephritis

79
Q

Bruton’s Agammaglobinemia

A

X linked; normal T-cells, but low to absence B- cells. Block between Pre-B and Immature B-cell(with IgD or IgM on surface)

80
Q

Dermatitis herpetiformis

A

skin manifestation of celiacs, IGA to TG3 in skin

81
Q

Autoimmunity Thrombocytopenic Purpura - symptoms

A

Bleeding abnormalities in young, healthy post viral infection or Older patient with autoimmunities; or from certain drugs

82
Q

HyperIgM Syndrome

A

Normal IgM, but can’t class switch to IgG or IgA (low levels); X-linked

83
Q

will the bump be hard or soft for a positive PPD test?

A

Hard - full of macrophages (type I) due to Th1 recruiting thousands of macrophages)

84
Q

Chron’s

A

CFR: large and small intestine, esp terminal illeum, patchy microabcesses that become granulomas

85
Q

Ataxia Telangiectasia signs and symptoms

A

AR; sinus infections, pneumonia, ataxia, telangiectasia (dilated abnormal vessles), tumors

86
Q

Rescue inhalers

A

short acting Beta2 antagonists - albuterol

87
Q

Type III in joints

A

athritis

88
Q

IgA Nephropathy

A

Type 3,asymptomatic, but presents with hematuria. IgA nephropathy causes terminal sugars in hinge to go missing and this causes cross reaction with normal IgA and IgG in renal glomerulus.

89
Q

Type III Peritoneum

A

Sterile peritonitis

90
Q

Why don’t we use oral polio virus vaccine anymore?

A

because people who have Bruton Agammaglobulemia are more susceptible to poliovirus (and all enterovirus) that is associated with the live virus.

91
Q

Common characteristics of all immunodeficiency diseases

A

Diarrhea and malabsorption of nutrients

92
Q

T-Cell Testing

A

Skin test to recall Ag panel, Total lyphocyte count, CD3, CD4, CD* THEN mitogen response, mixed leukocyte reaction, cytokine measurements, sequence suspect gene

93
Q

Adenosine Deaminase Deficiency (ADA)

A

adenosine accumulate in cells to impair lymphocyte development; AR form of SCID

94
Q

Psoriasis

A

CFR, Th17 dominated, associate with HLA-Cw*06:02

95
Q

Autoimmunity Hemolytic Anemia

A

Type II; Ab against RBCs usually post viral infcetion, with other autoimmune, cancer, or drugs

96
Q

primary immunodeficiency

A

mutations in gene required for normal development of immune system

97
Q

Chronic Beryllium Disease

A

(Type IV) Reaction of Be to cause scarring of lung tissue; related to HLA-DP1

98
Q

Post-Strep Glumerulonephritis

A

Type 3, 10-14 days post infection with strep, scarlet fever, impetigo of skin - NV, fever, malaise, hypertension, reduced urine output, hmaturia, joint paint and rash. disease is self limiting

99
Q

X-linked SCID

A

SCID-X1

100
Q

Type I diabetes

A

Type IV, autoab attack to beta islet clls in pancreas; HLA DR3 and DR4 are in linkage disequilibrium with DQ2 and DQ8 so they more readily bind to islet cells and present as foreign

101
Q

PPD on tests with BCG vaccine?

A

will also be Positive; must instead use Quantifiro test

102
Q

IgE production

A

Tfh/IL4 dependent. Slow process and requires multiple exposures.

103
Q

What was David - Bubble Boy’s treatment?

A

mismatched sisters BM with anti CD4 and CD8 and compliant to rid of mature T-cells to eliminated graft vs. host reaction

104
Q

Treatment of DiGeorge

A

Fetal thymus or thymus stromal transplant (at least 1 class I and Class II)

105
Q

Cross-Reaction

A

method in Type II immunopathy; between foreign antigen and self antigen

106
Q

Mechanisms of loss of tolerance in Type II immunopathy

A

Hybrid; Forbidden clone; Cross reaction; Passive ab; innocent bystander; release of sequestered antigen; failture of regulatory mechanism

107
Q

First Graft Rejection

A

10-20 days

108
Q

GoodPasture’s Syndrome - Symptoms

A

Glomerulonephritis, pneumotis, pulmonary hemorrhage

109
Q

Forbidden clone

A

method in Type II immunopathy; autoreactive T-cell escape

110
Q

Myasthenia Gravis - Mechanism

A

Type II immunopathy, Aire, a TF, doesn’t promote the synthesis of CHRNA1, which codes for alpha AchR, so AChr is not expresse din thymus and negative selection does not occur. T-cells then activate B-cells to release Ab against AChR in NMJ

111
Q

Complement Testing

A

CH50, assay for CH1inhibitor, THEN individual complement component levels

112
Q

Dressler’s Syndrome - Pathophys

A

Type II; AutoAb to pericardial of myocardial antigens

113
Q

Two phase of type 1 immunopathology

A

Early: histamine and can be treated with antihistamine, late: 4-10 hours laters, Prostagalingin, Luekotriene and cytokine release in inflammaotry. Treated with antiinflammaotry

114
Q

Chronic Beryllium Disease

A

CFR: inhaled Be covalently links to peptides to create neoepitope that riggers Th1 and later Th2. Cant be removed by macrophages. Chronic after 1 exposure. Linked to HLA-DP allel to create pocket for Be+

115
Q

Rheumatic Fever

A

different from Rheumatic heart disease in that it involves the Skin and CNS

116
Q

Lab values of CVID

A

Serum IgG

117
Q

Treatment of Type I immunopathology

A

avoid allergn, antihistamine, epineprine, steroids, leukotriene inhibit, long acting beta-2 antagonists, IgE blockers, immunotherapy

118
Q

Myasthenia Gravis - Symptoms

A

Progressive Muscle Weakness

119
Q

Late phase IgE reaction

A

PLA2 cleaves AA to make prostaglandin and luekotrienes, (4-10 hours later).

120
Q

Immediate IgE reaction

A

IgE binds to mast cells and basophils with IgE receptor FCeR1. high binding affinity. IgE relelases granules when two adjacent IgE molecules are cross linked by allergen.

121
Q

Selective IgA Deficiency

A

Most common immunodeficiency 1/500; mostly asymptomatic, bu can cause diarrhea, sino-pulmonary infection, increased severity of allergies; 10-15X more common in celiac patients

122
Q

Celicas Mixed

A

2: ab to tissue transglutaminase; CFR

123
Q

Biopsy of Type I Infections

A

infiltration of Th2 and eosinophils

124
Q

LABAs

A

long acting beta 2 agnosts - reduce bronchoconstriction for 12 hours - advair

125
Q

IBD

A

CFR: Genetic component with NOD2 (A PRR), but alos due to increase permeatbility of gut so secreted defense resturns to gut to activate DAMPs to increase IL6 and more TH1,2,17 against gut flora.

126
Q

Secondary immunodeficiency Drugs

A

Corticosteroids, monoclonal antibodies

127
Q

B-Cell Testing

A

Serum protein electrophoresis, quantitative IgG, IgA, IgM, sepcific Abs prior to immunization, ABO isohemagglutinins BEFORE Ab response to noveal Ag, sequence, lymph node biopsy

128
Q

Type IV Immunopathy - basics

A

Delayed Hypersensitivity reaction; T-cell mediated - does not require Ab

129
Q

SLE mixed

A

2: AutAb to dsDNA; 3: IgGdsDNA complex in kidney; 4: T cell mediated damage

130
Q

Conditions of Graft vs. Host

A

Graft must have T-cells, by antigenic, and be in an immunocompromised host

131
Q

Mixed RA

A

TYpe II: IgM against own IgG(Rheumatoid factor); Type III: trapped IgM and IgG complex; Type IV: T_cell mediated damage

132
Q

Passive Antibody

A

method in Type II immunopathy; hemolytic disease in newborn, mismatched transfusion, child of mother who has myasthenia gravis or SLE

133
Q

Lab findings of SCID

A

Lymphopenia in Both B and T cells; thymus shadow on X-ray, small tonsils, low mitogen response, low serum Ig

134
Q

UC

A

CFR: more superficial, in large intestine, leads to bleeding

135
Q

Wiscott-Aldrich

A

X linked; platelet and B-cell cell deficiency, eczema and bacterial infection

136
Q

HLA-B*5071

A

Abacavir hypersensitivity; in linkage disequilibirum with actual causal HLA-D gene. a Class I gene, so it elicits a CTL problem

137
Q

Goodpasture’s Syndrom mechanism

A

Type II immunopathy; AutoAb to Type IV collagen found in lung and kidney of Basement Membrane. Appears linear, not lumphy and bumpy with immunofluorescence

138
Q

what inflammatory mediators are involved in Type III

A

H2O2, proteinases, histamine

139
Q

Periodontal Disease

A

CFR major tooth loss, bacterial live in giginval crevice where T-cells can’t reach. Shift to increase IL-6/TGFbeta and we see Ab to citrullinated proteins lik in RA.

140
Q

Treatment of SCID

A

BMT (50% success rate); better to use purified hematopoetic stem cells

141
Q

Graft vs. Host

A

Type IV; acute (2-10 weeks; maculopapular rash, diarrhea, hepatic inflammation, infection) or Chornic (months to years) or vs. Leukemia.

142
Q

LIPT

A

sublingual immunotherapy for ragweed allergies

143
Q

Asthma

A

Type I, bronchoconstrictive iflammaotry, need to treat to avoid lung fibrosis

144
Q

Diagnosis of Type II immunopathologies

A

Direct or Indirect Immunofluorescence

145
Q

V(D)J

A

defect in SCID that is most common in navajo and apache children

146
Q

Treatment of Type I immunopathology

A

Avoidance, Antihistamines, epinephrine, steroids,Luekotriene inhibitors, rescue inhalers, long acting beta2 agonists, IgE blockers, Immunotherapy

147
Q

Eczema

A

Atopic derm, chronic dry and irritated skin, rash, self worsening (itching release more inflammatory cytokines), secondary infect common Type I; cross reactions are common

148
Q

Hypersensitivity Penumonitis

A

Type 3 Farmers lung, exposure to filamtenous bacteria in modly hay that causes serum IgG. when inhaled enough, forms complex and auses shortness of breath, dry gcough, malaisem tachycardia. if progessive, goes to type IV. treatment with steroids and avoidance

149
Q

Th2

A

helps switch B Cells ot make IgE. Release IL4, IL5, IL13 to activate M2 macrophages to heal damage and wall off.

150
Q

what is important to look for physical exam for immunodefiencies?

A

Development, tonsils, lymph nodes

151
Q

SCID - name

A

Severe Combined Immunodeficiency Disease

152
Q

Nude Mice

A

different mutation, but also fail to develop thymic stroma and have same characteristics of Digeorge

153
Q

Phagocyte Testing

A

WBC count, differential, morphology, NBT test, oxidative burst THEN assay for phagocytosis, chemotaxis, sequence

154
Q

Hyper IgE Syndrome

A

AD, inability to make IFN gamma (jobs Syndrome) which leads to Th2 dominance over Th. High serum IgE leads to skin abcesses, fungal or pseudomonas pneumonia

155
Q

Paraiste resonse

A

Type 1: IgG binds to worm or ova and activates complement, C3a and C5a attract neutrophils but nothing happens. IgE diffuses to nearby Mast cells and release histamine for smooth muscle contraction and peristalsis. Eosinophils are activated by IgG to release granules of Major Basic Proteins to kill worm.

156
Q

Antigens in type III

A

must be present in sufficient amount to form complexes (or depot of antigen)

157
Q

Steps in Type IV Immunopathy

A

Initiation: APC presents to Tcells, but not fast enough to generate reaction; Elicitation: T-cell response with peak of 24-48 hours due to memory T-cells

158
Q

Transient Hypogammaglobulinemia of Infancy

A

from 3-6 months up until 18th month; IgG production is low and results in recurrent and persistent gram positive infections, 15% of infant diarrhea

159
Q

Atopic state

A

predisposition to allergies

160
Q

Abacavir reaction

A

(Type IV) reaction that causes rash, malaise, variable GI, Respiratory tract, MSK reactions. Due to HLA-B*5071

161
Q

Lawsonine

A

good type of henna with no DTH

162
Q

Failure of Regulatory Mechanisms

A

method in Type II immunopathy; imbalance in type of T-cells? still undecided whether pathologic or therapeutic

163
Q

CH50

A

Type 3, total hemolytic complement levles. Pt serum is diluated and added to antibody coateded RBCs, maximal dilution to causes 50% of cells to lyse compared to standard.