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USLME Kiss my ass and pray > Immunology w/ DIT > Flashcards

Immunology w/ DIT Flashcards

1
Q

chemotactic factors for neutrophils (4)

A

Il-8 and C5a, LTB4, Kallikrein

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2
Q

Granuloma formation -

2 cells involved and cytokines (2)

A

Th1 secretes in IFN gamma activating macrophages.

TNF alpha from macrophages induce and maintain ganulomas

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3
Q

C3b

A

opsonization w/ IgG
also clears immune complexes

b binds

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4
Q

anaphylaxis complement products

A

C3a and C5a

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5
Q

Acute inflammation mediated by what cell

A

Neutrophil

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6
Q

Chonic inflammation medicated by what cell

A

Monocytes-> macrophages

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7
Q

Acute phase of inflammation initiated by what 3 cytokines

A

IL1
IL6
TNF alpha

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8
Q

Granuloma formation is dependent on this cell and its secreted cytokine

A

Macrophage and TNF alpha

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9
Q

4 Steps of Leukocyte extravasation

mediated by what receptors

A

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

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10
Q

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

deficient in what?

A

Leukocyte adhesion deficiency syndrome

deficient in integrins

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11
Q

cells that activate macrophages and cytokine

A

Th1 release IFN gamma to activate macrophages and granuloma formation

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12
Q

Granuloma diseases(11)

A 
TB
Fungal
trepona palliudum
M leprae
Bartenlla henslae (cat scratch fever)
Sarcoidosis
Crohn's disease
wegeners
Beryllosis, silicosis
Foreign body disease
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13
Q

C reactive phase vs sed rate

A

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

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14
Q

Vasodialation and vascular permeabilty in inflammation due to (3)

A

Histamine
serotonin
bradykinin

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15
Q

Fibroblasts needs what to lay down collagen

A

Vit C needed for fibrosis

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16
Q

Tissue remodeling needs what dietary molecule

A

Zinc

- done by metalloproteinases

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17
Q

12-24 hrs post wound see

what changes 2 days later

A

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)

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18
Q

Remodeling of the wound occurs when and what is done

A

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

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19
Q

B cells are located where in the lymph node?

A

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

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20
Q

T cells are located where in the lymph node

What else is located here

A

in the paracortex

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

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21
Q

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

A

Medullary cords - mature lymphocytes and plasma cells

Medulary sinuses w/ macrophages

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22
Q

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

What congenital deformity inhibits this?

A

T cells in the PARACORTEX

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

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23
Q

Functions of the lymph node?(3)

A

filtration w/ macrophages

storage and activation of B and T cells

Antibody production

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24
Q

secondary lymphoid organs (4)

vs primary (2)

A

lymph node, spleen, MALT and cutaneous lymph tissue

Thymus and bone marrow

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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
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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) ```
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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) ```
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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
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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)
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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

USLME Kiss my ass and pray (33 decks)

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