Immunology w/ DIT Flashcards Preview

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Flashcards in Immunology w/ DIT Deck (230):
1

chemotactic factors for neutrophils (4)

Il-8 and C5a, LTB4, Kallikrein

2

Granuloma formation -
2 cells involved and cytokines (2)

Th1 secretes in IFN gamma activating macrophages.

TNF alpha from macrophages induce and maintain ganulomas

3

C3b

opsonization w/ IgG
also clears immune complexes

b binds

4

anaphylaxis complement products

C3a and C5a

5

Acute inflammation mediated by what cell

Neutrophil

6

Chonic inflammation medicated by what cell

Monocytes-> macrophages

7

Acute phase of inflammation initiated by what 3 cytokines

IL1
IL6
TNF alpha

8

Granuloma formation is dependent on this cell and its secreted cytokine

Macrophage and TNF alpha

9

4 Steps of Leukocyte extravasation
mediated by what receptors

Rolling - E selection on endothelial cells (P selection and L selection in leukocytes)

Tight binding - ICAM1 on endothelial cells and LFA1 (integrins)on PMNs

Diapedesis - PECAM on both cells

Migration - no recptors, follow chemotactic signals

Whole process enhanced by platelet activating factor

10

Patient presents w. delayed separation of the umbilicus and has recurrent bacteria infections is suffering from what ?

deficient in what?

Leukocyte adhesion deficiency syndrome

deficient in integrins

11

cells that activate macrophages and cytokine

Th1 release IFN gamma to activate macrophages and granuloma formation

12

Granuloma diseases(11)

TB
Fungal
trepona palliudum
M leprae
Bartenlla henslae (cat scratch fever)
Sarcoidosis
Crohn's disease
wegeners
Beryllosis, silicosis
Foreign body disease

13

C reactive phase vs sed rate

both non specific election in inflammation -

Sed rate is fibrinogen coated RBC settle faster
- > increased Sed rate (helpful in osteomyolytis) infection, inflame, CA, pregnancy, SLE, RA

- low in Sickle cell and polycythema

C reactive protein is an acute phase reactant made in the hepatocyte

14

Vasodialation and vascular permeabilty in inflammation due to (3)

Histamine
serotonin
bradykinin

15

Fibroblasts needs what to lay down collagen

Vit C needed for fibrosis

16

Tissue remodeling needs what dietary molecule

Zinc
- done by metalloproteinases

17

12-24 hrs post wound see

what changes 2 days later

acute inflammation and PMN -increase vessel permeation

Macrophages come in and assess
- fibroblast, myfibroblast and endothelial cells and epithelilaization (skin and wound coverage for 28Hrs)

18

Remodeling of the wound occurs when and what is done

1 week after and fibroblasts change type III (granulation tissue) collagen to Type I (scar)

19

B cells are located where in the lymph node?

in the follicles located in the cortex - B cell localization and proliferation

20

T cells are located where in the lymph node

What else is located here

in the paracortex

The high endothelial venules (HEV) are also located here where T and B cells enter from the blood

21

What is located in the medulla of the lymph node (2)

Medullary cords - mature lymphocytes and plasma cells

Medulary sinuses w/ macrophages

22

What region of the lymph node expands greatly during a viral infection?

What congenital deformity inhibits this?

T cells in the PARACORTEX

DiGeorge -> lack of thymus -> lack of t cells

23

Functions of the lymph node?(3)

filtration w/ macrophages

storage and activation of B and T cells

Antibody production

24

secondary lymphoid organs (4)

vs primary (2)

lymph node, spleen, MALT and cutaneous lymph tissue

Thymus and bone marrow

25

MALT tissue is located (4)

lamnia propia of the intestine -GALT

adenoids/tonsils (o-malt)

upper airways (BALT)

nose associated lymph

26

Payers Patches differ from lymph nodes in that?

Located where?

they are unencapsulated and located in the lamina and sub mucosa of the illiim of the smallintestine

27

M cells function in peters patches

flattened areas that take up antigen, transcytosis it, allows APCs to take up to the geminal centers deeper and allow IgA to be made in prevention of disease

28

Right lymphatic duct drains?

vs Thoracic Duct

R arm
R chest
R half of face

Drains everything else located at the L subclavian and internal jugular vein

29

Extremities Lymph Drainage

Lateral side of dorsum of the foot drains?

Thigh drains?

Upper limb and lateral chest

Lat foot drains to popliteal

Thigh drains to superficial inguinal

Upper limb and Lat chest drains to axilla (next is parasternal/mammary nodes)

30

GI Lymph drainage

Stomach drains to ?

Duodeum/ illium?

Sigmoid Colon?

Stomach -> celiac

Duodeum and illieum -> superior mesenteric

Sigmoid colon -> colon-> inferior mesenteric

31

Inguinal/Rectal lymph drainage

Rectum drains?

Testes drain?

Scrotum drains?

Rectum above the pactinate line -> internal iliac

Rectum below drains to superficial inguinal (epithelial derived)

Testes drain to pre-aortic*
- (back to where derived)

Scrotum drains superficial inguinal

32

Innate immune response consists of? (8)

neutrophils
macrophages,
NK cells - lymphoid derived*
Dendritic
Mast cells
eosinophils
basophils

Complement protein (MAC)

33

Adaptive immune system response consists of ? (3)

B cells
T cells

Circulating antibody

34

CD 3

cell marker on all T cells (helper and cytotoxic)

35

the majority of lymphocytes in circulation (80%) are

T cells

Also refers to B cells

36

MHC Class I is recognized by?
- binds to?(2)

Made from what genes?

CD8 Cytotoxic T cells
- TCR and CD8
- Viral and CA immunity

HLA - A, HLA - B, HLA-C

37

MHC class II is recognized by
- binds to ? (2)

Made from what genes?

CD4 helper t cells
-TCR and CD4
- Extracellular antigen response

HLA - D
- DR or DP or DQ

38

MHC I is expressed on

All cells in the body except RBCs

39

MHC II is expressed on

APCs (3)

Dendrites
Macrophages
B cells

40

HLA - B27 assoicated w PAIR

Psoriasis
Ankylosing spodylitis
IBD
Reiters Syndrome

41

DM and graves and DM and RA is associated w/ what HLA type?

DR3

DR4

42

Dendritic cells have 4 receptors on them

MHC I - all Cells
MHC II - APC
B 7 (CD80 or 86) - co stim
CD40 - activated by T cells

43

Dendritic cells are located (2 locations)

Langerhorns - in the skin

interstitial dendritic cells - minus the brain

44

Dendritic cells sample for antigens via (3)

phagocytosis
pinocytosis
receptor mediated endocytosis (clathrin mediated)

45

Birbeck granule

Cells expired what tumor markers? (2)

tennis racket shape langerhan cell implicated in langerhan cell histiocytosis

S-100 - nueral crest cell origin
CD1a

46

Kid presents w/ a lytic bone lesions and a skin rash - biopsy shows cells that normally express B7, CD40, MHC 1 and MHC 2 markers

What is expected on EM

langerhans cell histiocytosis - a bunch of dendritic cells that do not work

- now expressing S-100(nueral crest cell origin) and CD1a

see birbeck cells, tennis rackets on EM

47

Immature T cells are found where?

What do they undergo to become mature T cells

in the cortex of the thymus

Undergo + selection in the cortex and - selection in the medulla as they make their way to the medulla of the thymus

48

cells in the cortex of the thymus express what markers (3)

CD3, CD4 and CD8

49

positive selection means what for a T Cell

When CD8 and CD 4 receptors are both expressed and binding to a antigen presented by the thymic cortex leads to differentiation

CD8 selected if bound to MHCI

CD4 if bound to MHCII

50

Negative selection for a T cell means

in the medulla T cells that bind to tightly to self antigen are selected against for apoptosis

51

During a viral infection what cytokine is present in the lymph node that will lead a nieve T helper cell down a particular path?

IL12 leads to Th1 cell differentiation and further activation of Cytotoxic T cells and Macrophages through IL 2 and IFN gamma

IL 12 comes from macrophages

52

During an extracellular attack what cytokine released leads to a nieve helper T cell become a helpful cell in the attack

IL4 leads to Th2 differentiation and further activation of B cells through the release of IL2, IL4, IL5, and IL10

53

Th1 is suppressed by what cytokine

IL10

also blocks macophage activation

54

Th2 is suppressed by what cytokine?

IFN gamma

also activates macrophages

55

IL 2 role in infection

released by both Th1 and Th2 and leads to T cell proliferation
Th1 and CD8 in cell mediated or Th2 in humoral

56

2 Signals needed to activate nieve T cell

1. MHC class II to TCR on CD4 Th1 or Th2
2. CD28 w/ B7 (CD80 or 86)

-leads to IL2 and monoclonal T cell proliferation

1.*MHC I if activating a CD8
and 2. CD 28 w/ B7(CD 80 or 86)

57

CD 28

Co stimulatory factor found on T cells that are activated / B 7 (CD80 or 86) from an APC

58

B cell Activation signals (2)

MHC II on B cell goes to a Th2 cell and presents as APC

CD40 L on Th2 binds w/ CD40 on B cell to co-stimulate and activate the B cell

- Cytokines IL4 or IL5 class switch the B cell appropriately

59

Th1 secretes (2)

IL 2 - t cell prolif

IFN gamma - negative feedback on Th2 and activates macrophages

60

Th2 secretes (4)

IL-2 - t cell proliferation

IL 4 - class switch and + feed on Th2

IL 5 - class switch

IL10 - neg feedback on macrophage and Th1

61

Cytotoxic T cells kills cells by (2)

Releasing perforin and granzyme
-also done by NK cells

Binding FAS L w/ target FAS receptor

Induing apoptosis

62

CD8 cells have a role in defense against (3)

Viral
CA
transplants

-faulty MHC I presentations

63

Regulatory cells release?(2)

Gene expression and associated receptor

Anti inflammatories and IL10 and TGF beta

FOXP3 -> CD25
(also has CD3 and CD4)

64

NK cells activity normally is?

enhanced by?

killing of cells w/out MHC I through perforin and granzyme

lymphocyte origin*

Activity enhanced by IL2, IL12, IFN beta and IFN alpha

65

IFN alpha and Beta in a viral infection

warns nearby cells to shut down viral producing products and up regulates MHC I expression

differs from IFN gamma

66

CD16

found on NK cells, macrophages, and PMNs,

Recognizes Fc component of antibodies and used in antibody dependent cell mediated cytotoxicity (ADCC) - opsonization

67

CD56

cell marker for NK cells

Also has CD 16

68

Only lymphocyte of the innate immune system?

NK cells

69

B cell Surface Markers (8)

CD19
CD 20
CD 21 - EBV

CD 40 - T cell gives permission to activate

MHC II - APC
B7 - Co-stim B activates T cell

IgM
IgD

70

Heavy Chain of Antibody chains in an Antibody important in determining what?

Isotype of the antibody

IgM - mu
IgG -gamma
IgA - alpha
IgE - epsilon
IgD - delta

71

2 ways to describe an antibody

Light Chain/Heavy chain

or Fab and Fc portion

72

Light chain of antibody is important in determining what?

A part of the Fab portion that leads to a unique antigenic pocket (idiotype)

2 types Kappa and lambda;
2:1 ratio normally- multiple myeloma if not

73

What ratio is kappa light chin suppose to be to lambda light chain?

2:1

multiple myeloma if not

example should be gamma 2 lambda 2

74

Fab portion of the antibody is important for?

Determining the idiotype - unique

75

Antibody light chain and heavy chains are held together by

di sulfide bonds

76

Fc portion of the antibody is important for (2)

Constant portion

complement binding - At Ch2 (IgM and IgG)

Determining the isotope

77

What initiates recombination of the V(D)J sequence

Gene sequence recognized?

Recombination Activating Gene complexes (Rag 1 and 2 ) recognize Recombination Signal Sequences (RSSs) that flank V(D)J coding sequences and result in breaks of dsDNA and antigenic uniqueness from somatic hypermutation

78

Mutations in RAG genes results in what

Inability to initiate antigenic reombination sequences of the VDJ sequence

Can't recognize the RSSs

79

Antibodies have 3 roles in the body

Obsonization - (phagocytosis)

Neutralization - (toxin or bacteria itself)

Complement activation - MAC

80

Fc of an antibody determines the?

isotype

81

Fab of an antibody determines the

idiotype

82

Mature B cells express on their surface what Igs?

IgM and IgD

83

Main secondary antibody found evenly distributed in the intravascular and extrvascular pools

IgG

84

4 jobs of IgG

fixes complement

Crosses the placenta to provide passive immunity

opsinizes bacteria

neutralize bacterial toxin/viruses
-mediates cell mediated cytotoxicity

85

Antibody that crosses epithelial cells by transcytosis?

Covered by what?

IgA

Covered by secretory component,

Found in secretions (tears, saliva, mucus) and breast milk

86

IgA exists in 2 forms

monomer in circulation

dimer in secretion _ attached at Fc part

87

IgE is assocaited w/ 2 processes

1. Immunity from worms w/ activation of esinophils

2. binding to mast cells and predisposing for hypersensitivity type 1 reactions through histamine release

88

Antibody produced in immediate response to an antigen

Exists in 2 states

IgM

Pentomer in circulatory -> wheel (too big to cross the placenta)

monomer on surface of B cell

89

2 antibodies that can fix complement

IgG and IgM

90

B cells are encouraged to class switch with what 2 signals

Cytokine IL 4 or 5

CD 40 stim from CD40 L (T cell)

91

Be wary of giving the following 2 vaccines to someone with an egg allergy

Yellow fever

Influenza

92

Examples of live attenuated vaccines (6)

Induces what kind of response?

Measles
Mumps
Rubella
Varciella
Polio - Sabin (oral)
Yellow Fever
Intranasal flu

Induces a cellular response w/ cytotoxic t cells - actually infects the cell and leads to memory cells

93

humoral immunity is induced by what type of vaccine

Examples(5)

killed or inactivated vaccines, only elite b cell response

polio - salk (IM)
Hep A
Rabies
Polio
IM flu

94

3 vaccines still recommended for HIV patients if their CD4 counts are above 200

MMR
Varicella
Yellow fever

95

Thymus independent antigens are?

Leads to

An example?

antigens lacking a peptide component and thus cannot be presented by MHC to T cell-> NO immunologic memory, just some IgM

LPS is poslysachride and not a protein - needs to be combined w/ a protein like diphtheria to induce a cytotoxic response to gram - bacteria

96

What is needed of an antigen to initiate class switching of a B cell?

a protein which can presented on an MHC class II cell

-> CD 40 (b cell) and CD 40L (t cell) binding -> response

97

Amyloid deposited in response to a plasma cell disorder or multiple myelinoma

AL

otherwise known as primary amyloidosis

98

Amyloidosis stain and responding color?

congo red stain

apple green birefringence

99

Amyloidosis def

abnormal aggregation of proteins or their fragments into beta pleated chest structures

waxy appearance of the tissue as a result

100

Secondary amyloidois deposits?

Appears w?

AA due to accumulation of Serum associated amyloid

seen in chronic disease like Ra, IBD, spondyloarthopathy and chronic infection

101

Dialysis related amylodosis see?

Abeta2M deposit - kidneys don't eliminate

complaints of carpal tunnel and joint issues

102

Amyloid deposit seen in alzheimers?

Beta amyloid

from amyloid precursor protein (APP)

103

Auto IgG antibody

Rhumatoid Factor - RA

104

Anti citrullinated protein antibody

more specific RA marker

105

Anti centromere

CREST Syndrome

106

Anti SCL70

Diffuse Scleroderma

107

Anti histone

Drug induced SLE

108

Anti dsDNA

nephrotic SLE

109

Anti smith

SLE

110

Anti Jo1

Polymyositis/dermatomyositis

111

Anti SSA

Sjogrens

112

Anti U1 RNP

Mixed connective tissue disease

113

Anti desmoglein

Phemphigus vulgaris

114

Anti acytelcholine receptor

Myasthenia Gravis

115

Anti endomysial / anti tissue transglutaminase

Celiac

116

Anti gliadin

Celiac

117

Anti mitochondrial

Primary biliary cirrhosis

118

Anti smooth muscle

Auto immune hepatitis

119

Anti glutamate decarboxylase

DM1

120

Antithyrotropin receptor

Graves Disease - Stim

121

Anti Thyriod peroxidase (anti TPO)

Hashimotos

122

Anti thyroglobulin

Hashimotos or Graves

123

Anti basement membrane

Goodpastures

124

c-ANCA

Wegeners - Granulomatosis w/ polyangiitis

125

p-ANCA(3)

Pauci immune crescent glomerulonephritis

Churg Straus

Microscopic polyangitis

126

Type I Hyper sens characterize by

Presensitized mast and basophil cells w/ IgE bound to them react to antigens resulting in release of histamine and brady kinins -> vasodilatation and increased permeability

Ex; wheal and flare hives and anaphylaxis

127

Eczema is what type of hypersensitivity

Type I

128

Asthma is what type of hypersensitivity?

Type I

129

Type II hypersensitivity is characterized by?

Antibodies (IgM and IgG) binding to fixed antigens found on cells -> cell destruction(cytotoxic) by:

-opsinization
-Complement mediated lysis
- Antibody dependent cell mediated cytotoxicity

Ex: ABO and Goodmans

130

Rheumatic fever hypersensitivity ?

Type II

131

Goodpastures hypersensitivity?

Type II

132

Hemolytic anemia hypersensitivity ?

Type II

133

Graves hypersensiivity Type?

Type II

134

pemphigus vulgaris hypersensitivity type?

Type II

135

ITP immunologic thrombocytopenic purpura hypersensitivity?

Type II

136

Myasthenia gravis hypersensitivity Type?

Type II

137

Pernicious anemia hypersensitivity?

Type II

138

Type III hypersensitivity characterized by ?

Antibodies binding to soluble antgens in the serum and eventual deposition leading to 3 things

Activation of complement
attraction of neutrophils
local release of lysosomal products

Ex: SLE serum sickness, arthus reaction

139

Serum Sickness?

Type III hypersensitivity where antibodies react to happens (protein bound drugs) resulting in insoluble complexes that deposit and promote local immune response - TAKES TIME

140

Arthus Reaction?

Local Type III hypersensitivity reaction where injected antigen binds to antibodies and lead to activation of complement -> necrosis, edema.

Example: Rxn to genus shot - IMMEADIATE w/in hrs vs Type 4 PPD which takes 24-48hrs

141

SLE hypersensitivity reaction?

Type III

142

Rheumatoid Arthritis hypersensitivity reaction?

Type III

143

Polyarthritis nodosa hypersensitivity reaction?

Type III

144

Streptococcal glomerulonephritis hypersensitivity reaction?

Type III

145

Type IV hypersensitivity reaction characterized by?

DELAYED - cell mediated T cell response to encountered antigen where cytokines (IFN gamma and IL2) are released to recruit macrophage and have local response

takes 24-48 hrs

Ex: PPD, contact dermatitis, hashimotos

146

Hashimotos hypersensitivity reaction?

Type IV

147

Contact dermatitis hypersensitivity reaction?

Type Iv

148

PPD hypersensitivity reaction

Type IV

149

Guillaiane Barrie hypersensitivity reaction?

Type IV

150

Multiple Sclerosis hypersensitivity reaction ?

Type IV

151

3 ways of activating the complement system

Classic
- IgG/IgM binds to C1 -> response

Alternative
- Spontaneous or microbial surface -> C3

Lectin pathway
-mannose binding lectin(protein) binds to microbial surface

152

Complement factors making up the MAC complex,

Lack of them increases risk of?

C5b - C9

Nesseria infection

153

Important soluble immune components in opsonization

IgG

C3b (Binds to make tasty)

154

Important complement components in mediateing anaphylactic reaction

C3a

C5a (also important in chemotaxis)

It is usually the a subunit that floats away

155

Defect in what part of the complement system leads to hereditary angiodemema

Do not give what drug

C1 esterase inhibitor
- stops improper complement activation
-get C5a and C3a leading to anaphylactic reactions

Do not give ace inhibitors

156

Patient presenting w/ recurrent pyrogenic and respiratory bugs like Strep pneumo and haemophilus influenza may be deficient in what complement factor

Also at increased risk of what?

C3

Type III hypersensitivity

157

Paraoxysmal nocturnal hemoglobinuria is deficient in what?

Deficient in glycospphosphatididylliosital( GPI)
-an anchoring protein

NO DAF - CD55 or CD59
- prevents the improper hemolysis through complement activation

Rx Eculizumab

158

Hams test test that may be used when patient has increased RBCs in his urine especially in the morning

Paraoxysmal nocturnal hemoglobinuria

increased lysis in the presence of acid

Deficient in CD 55/59 DAF

159

CD 55 and CD 59

DAF - decay accelerating protein
- blocks complement activation

160

Thrombosis and hemosindeinurea due to

Paraoxysmal nocturnal hemoglobinuria

Deficient CD 55/59 - DAF

161

T cells are found where in the spleen

in the PALS periarterial lymphatic sheath near the central arteriole in the white pulp

P's
paracortex in lymph node PALS in spleen

162

Red pulp of the spleen contains

RBCs

163

Macrophage function in the spleen (2)

Location?

Degrades broken down RBCs

phagocitizes encapsulated bate ria that have been opsonized w/ IgG and C3b

Located in the marginal zone of the white pulp

164

Post splenectomy see what problems in with platelets and RBCs (3)

low platelets and RBCs - thrombocytopenia due to loss of storage site

also :
Target cells
Howell jolly bodies (nuclear remnants)

165

CD 14 found primary where and responsible for what?

Found on macrophages

Recognizing LPS endotoxin leading to the release of IL1, IL6, and TNF alpha from the macrophage

CD 40 on macrophages sensitize it to IFN gamma

166

Various jobs of the macrophages (6)

1. present antigen
2. phagocitize bacteria
3. Phagocitize cell debris, clean up wounds (RBCs)
4. Form multinuclear giant cells in grnaulomas
5. respond to obsinized bacteria
6. Generate free radices using NADPH oxidase

167

Granulocytes refer to what 4 cells

Neutrophils
Eosinophils
Basophils - found in blood
Mast cells - found in tissue

168

Eiosinophilia Differential

DNAAACP

Drugs
Neoplasm
Acute interstitial nephritis
Adrenal insufficiency (Addisons)
Allergies/Asthma
Collagen vascular disease (PAN, dermatomyolitis)
Parasites

169

Monocyte derivatives(7)

leave the bone marrow to become mature in 8 hrs aftr migration to target

Joints - A cells
Blood.alveoli/intestines - macrophages
Connective tissue - histiocytes
Liver - kupfer cells
Kidney - mesangial cells
brain - microglial
bone - osteoclasts

170

A cell

joint monocyte

171

macrophage

blood/alveoli.intestine monocyte

172

histiocyte

Connective tissue monocyte

173

Kupferr cell

liver monocyte

174

mesangial cell

Kidney monocyte

175

microglial

brain monocute

176

costeoclast

bone monocyte

177

Cromolyn sodium

prevent mast cell degranulation

- old asthma medication needed 3-4x a day

178

Cytokines released by macrophages(5)

IL1
IL6
TNF alpha

IL8 -> chemotaxic PMN

IL12 -> Th1

179

Cytokines released by T cells(6)

all
-IL2 - T cell prof
- IL3 ~GM-CSF

Th1
-IFN gamma

Th2
-IL4 -> IgE and IgG in B cells
-IL5 -> IgA and eosinophils
-IL10 -> inhibits Mo and Th1

180

Cytokine involved in the growth and activation of eosinophils

IL 5 from Th2

181

Pyrogens secreted by monocytes and macrophages

IL 1 and IL6

182

Cytokine that mediates septic shock

TNF alpha from macrophage -> leukocyte recruitment and vascular permeability

183

Acute phase cytokines mediating inflammation

IL 1, IL 6, TNF alpha from macrophages

184

Enhances synthesis of IgA cytokine

IL 5

185

Enhances synthesis of IgE and IgG cytokine

IL4

186

Cytokines released by viral infected cells

IFN alpha and beta

187

Cytokine that supports bone marrow stem cells

IL3

188

Cytokine that supports T cell proliferation, differentiation and activation

IL2

189

IFN Alpha and beta encourages what cells to do what

uninfected cells near viral infected cells

encourages activation of ribonuclease that inhibits viral synthesis by degrading viral mRNA

190

IFN gamma leads to(2)

Increased MHC I and II expression

activates NK cells and macrophages

released by viral infected cells and Macrophages

191

Hyperacute transplant rejection occurs how quick?

Mediated by?

In min-hrs

Due to Type II hypersensitivity reaction due to preformed antibodies present in the serum of the recipient -> occluded graft vessels (necrosis and ischemia)

192

Acute transplant rejection occurs how quick

Mediated by?

Occurs w/in 1st 3 months
-usually weeks

Mediated by type IV hypersensitivity reaction - cell mediated response to the foreign MHCI -> vasculitis of graft vessels (complement activation)

193

Chronic transplant occurs how quick?

Mediated by?

Within months to years

Mediated by recognition of MHC I (non self receptors) as MHC I (self) presenting foreign antigens
-mix of cell mediated and antibody

Irreversible damage and fibrosis seen w. vascular damage

194

Pathogenesis of graft vs Host disease

Presents as?

Occurs when you transplant immune competent tissue into an immune compromised patient and the donated material reacts against the host

Presents as maculopapular rash(neck, shoulder ears and palms); hepatosplenomegaly; GI distress; hemolysis/jaundice

195

2 immune sup presents that work by blocking blocking Calcineurin

What does blocking ultimately do?

Cyclosporin
-binds to cyclophilin to make a complex

Tacrolimus
-binds to FK binding protein to make a complex

Both end up blocking the production of IL2

196

Known immunosupressant w/ nephrotoxicity(2)

associated side effect for both?

Tacrolimus

Cyclosporin
-gingival hyperplasia

HTN w/ decreased blood flow to the kidneys

197

Immunosuppresent that inhibits mTOR by binding to what that has a noted feature of NO nephrotoxicity

Sirolimus

Binds to FKBP12
->inhibits T cell response to IL2

198

Which drug has increased adverse side effects when given w/ Allopurinol?

Azathioprine
a 6 mercaptopurine precursor

Both drugs are metabolized by xanthine oxidase

199

Toxicity of azathioprine

Bone marrow suppression
- by excessive blocking of synthesis of nucleic acids (purine anti metabolite)

200

Autoimmune antibody that binds to CD3

especially useful for?

Muromonab CD3

immune suppression post renal transplant

201

Antibody that binds to IL 2 and blocking signal down steam -> decreased immune response

Daclizumab

202

Immune suppressor w/ increased lymphoma risk and has congenital defects w/ miscarriage risk as a list of side effects

mycophenolate mofetil

- inosine monophosphate dehydrogenase inhibitor blocking the creation of guanine

203

Immune supresor w/ history of phocomelia (severe both defects) and acts by blocking TNF alpha

Thalidomide

- immune suppression
-anti-angiogenic

204

Immuneosuppressent that binds to CD25

Result?

Daclizumab

CD 25 is IL 2 Receptor in activated T cells

205

Drugs used in lupus nephritis

Mycophenolate mofetil

Azathioprine*

206

X linked immune deficiency Disorders (4)

WACHS

Wiskott aldrich syndrome
brutons Agammaglobulinemia
+/- Chronic grammulomatos Disease
+/-Hyper IgM
+/- SCID (most common)

207

Patient presents w. recurrent bacterial infection 7 months after being born. No immunoglobulins noted

Defect due to absent?

X linked agammaglobulinemia
- Defect in BTK - a tyrosine kinase gene leading to no B cell Maturation

208

Brutons agammaglobulinemia

Presents as?(3)

Xlinked defect in tyrosine kinase gene

-Recurrent bacterial infections
-post 6 months
- Low Ig of ALL clases

209

Patient presents w/ recurrent sinusitis and pneumonia. Other than that the patient is fine

Be worried of what major complication

Selective IgA deficiency
- most common primary immunodeficiency
-Normal IgM, IgG

Be worried of anaphylaxis when given blood products w/ IgA

210

IgA is more commonly asymptomatice but more commonly seen w/ what disorders

presents w?

Atopic and asthma w/ elevated IgE maybe

Presents w/ recurrent pulmonary infections (mucosal infections)

Anyphylaxis to IgA containing blood products

211

Patient has a history of viral/fungal and protozoa infections w/ congenital heart defects.

What also may be low in the patient?

Low calcium in DeGeorges syndrome due to failure of Pouches 3 and 4 to develop

Tetrology of Fallot and transposition of great vessels may be seen

212

Digeorges syndrome presents immune deficiency due to lack of what cell type?

Predisposed to what infections

Thymus is gone -> no T cells

At risk for viral, proposal and fungal infections

213

Patients that are at increased risk for mycobacterial infection due to this inherited immune defect?

Pathogenisis?

IL12 receptor deficiency

-> low Th1 response -> low IFN gamma -> low granuloma formation

214

Patient presents w/ recurrent Candidiasis infection due to a congenital deficiency called?

defect is in what cell?

Chronic mucocutaneous deficiency

Defect in T cell, give ketoconazole

215

Patient presents w/ failure to thrive, chronic diarrhea and recurrent bacterial, viral, protozoa and fungal infections due to?

What is the primary defect?

SCID - severe combined immunodeficiency


Primary defect is usually Auto Recessive mutation which is adenosine deaminase deficiency -> toxic metabolite build up for T cells

Can also be X linked defect in IL2

216

Absence of a thymic shadow may be seen in 2 immune deficiency states

Digeorges
SCID

217

NO B or T cells are found in this disorder and NK cells are the only remaining response

SCID

218

Patient presents w/ gait disturbance and lack of smooth tracking movement w/ eyes and also recurrent sinopulmonary infection?

Increased risk for?

Ataxia telangiectasia
- see also telangiectasia
-IgA deficient

Risk of lymphoma and leukemia
- due to ATM defects which code for DNA repair mech- Don't X ray

219

Ataxia telangictasia triad

Due to?

1. Ataxia (cerebellar defects, smooth eye movement)
2. telangiectasia - on the face >5
3. IgA deficiency

Due to ATM mutation which codes for DNA repair (lymphoma risk) - Also have increased AFP

220

Increased AFP after 8 months seen in this IgA deficiency disease

Ataxia telangictasia

221

Defect seen w/ X linked associated damage to CD40L on helper T cells leads to

Hyper IgM syndrome
-Low IgG, IgA, IgE

Can also be due to AR CD 40 absent on B cells

222

Severe pyrogenic infections and lack of lasting immunity due to no class switching can be due to

Lack of CD40 L function in Hyper IgM syndrome

223

Wiskott Aldrich syndrome presents w?(3)

Defect?

Thrombocytopenia w/ Purpura
Eczema - on trunk
recurrent pyrogenic infections(decreased IgM compared to IgA against capsular polysachraides)

X linked Defect in WAS gene -> unable to reorganize actin skeleton

224

Recurrent pyrogenic infections and persistent itchy rash on the trunk can be due to?

Amy see what with blood drawn?

WAITER

Wiskott
Aldritch
Immunodeficiency
Thrombocytopenia w/ purpura *
Eczema - trunk
Recurrent pyrogenic infections (low IgM, higher IgA)

225

Defect in CD 18 leads to what primary immunodeficiency?

Presents as?

Leukocyte adhesion deficiency

Presents ad recurrent bacterial infections W/OUT pus formation

Lack of LFA 1 integrins to mediate leukocyte transmigration - Neutrophilia in the blood

226

Delayed separation of the cord may indicate what future problems for the infant

Lack of CD18, LFA1 integrin leading to Leukocyte adhesion deficiency

Recurrent bacterial infections

227

large lysosomal vesicles in phagocytes

Chediak Higashi syndrome

- defect in lysosomal tracking leading to lack of phagosome lysosome fusion

lysosomal regulator gene LYST defective -> microtubule dysfunction

228

Triad of partial albinism and peripheral neuropathy(ataxia or seizures) may hint at what primary immune deficiency?

Primary defect in that deficiency

Chediak Higashi syndrome

Microtubule dysfunciton in phagosome and lysosome fusion
- Auto recessive

229

What is missing in chronic granulomatous disease?

Presentation

NADPH oxidase which leads to the formation of ROS for destruction w/ respiratory oxidative burst

Recurrent infections w/ catalase + infections seen (S aureus, E coli, Klebsiella, Aspergillus, Candidia, Salmonella)

230

Nitoblue tetrazolium dye test does not turn from yellow to black is indicative of what primary immune deficiency?

Chronic granulomatous disease w/ no NADPH oxidase activity

X linked