Nelson

Question 1

What is the underlying pathologic mechanism of SLE?

Answer 1

Anti-nuclear antibodies–> immune complexes (3)

• smith and dsDNA abs are dx
• there is some type 2 as well

Question 2

What does anti-phospholipid abs in SLE cause?

Answer 2

Thrombosis- young stroke

• increase INR
• false + syphillis test
• can also lead to miscarriages and cerebral ischemia

Question 3

What is the underlying pathologic mechanism for RA?

Answer 3

Cyclic citrullinated proteins are dx

• infection and smoking lead to increase production of CCPs
• HLA-DRB1

Question 4

What is Sjogren syndrome?

Answer 4

Chronic dry eyes and dry mouth

• SSA and SSB not specific but you need lip biopsy to confirm dx
• most common in middle ages women
• can lead to marginal zone lymphoma

Question 5

What is the underlying pathogenesis of scleroderma?

Answer 5

Abs against Scl-70 (DNA topoisomerase)

• diffuse or limited
• CREST

Question 6

Dermatomyosisits vs polymyositis?

Answer 6

Elevated CK and treat with immunosuppressants

• Derm has discoloration under eyes with orbit edema
• poly- anti-Jo1 against muscle cells
• derm actived b and t cells leads to abs with complement

Question 7

What should one screen for with a pt with dermatomyositis?

Answer 7

Malignancy (15-25% of pts

Question 8

What 2 ag’s are most important in determining the likelihood of rejection?

Answer 8

HLA

ABO

Question 9

What is the difference between direct and indirect cellular rejection?

Answer 9

Direct is where the graft has the mediated response

- indirect is host APCs are responsible

Question 10

How about the humoral rejection?

Answer 10

Ab mediated

• preformed or develop after transplantation
• prior preg, prior transplant or blood transfusion

Question 11

what cause GVHD?

Answer 11

When a immunologically competent donor T cell recognize the recipients HLA as foreign ags

Question 12

What is AL amyloid light chain?

Answer 12

Accumulation of either Lambda or kappa light chain produced from free immunoglobulin light chain protein by plasma cells

Question 13

What is AA amyloid ass?

Answer 13

chronic inflammation

• SSA protein proteolysis
• SAA is an acute phase protein

Question 14

What is Beta amyloid?

Answer 14

From amyloid precursor protein

- A dz and cerebral plaques

Question 15

What is trasthyretin (TTR)?

Answer 15

transport thyroxine and retinal

- cause heritable nueropathic and cardiomyopathy amyloidosis

Question 16

what is B2 microglobulin?

Answer 16

cannot be filtered via dialysis

Question 17

What is the mechanism of light chain restriction?

Answer 17

In infectious dz we see polyclonal proliferation but in neoplasms we see monoclonol
- thus we see either kappa or lambda single ab increase in neoplasia

Question 18

What does reactive folicular hyperplasia have that a malignancy doesnt?

Answer 18

tangible body macs

Question 19

Type 1 hypersense?

Answer 19

IgE mediated and must have previous sensitzed to ag

• ATOPY- genetic predisposition
• late phase response
• fall in bp, bronchospasm and larnygeal edema

Question 20

Type 2 hypersense?

Answer 20

Ab mediated

• Linear deposition
• Myasthenia gravis or good pasture syndrome

Question 21

Type 3 hypersense?

Answer 21

Immune complex mediated

• Ab complexes deposit on endothelial wall
• kidney and synovium common site
• fibroid necrosis
• vasculitis, glomerulonephritis and arthritis

Question 22

Typer 4 hypersense?

Answer 22

Cell mediated

• T cell mediated and no ab
• Mantoux test- delayed type (CD4)
• DM1 and graft rejection mediated by CD8
• leads to granuloma formation
• caseating have central necrosis
• non case in chrons or sarcoidosis

Question 23

what are passenger mutations?

Answer 23

no phenotypic consequence

- occur during cancer growth but dont contribute to development of cancer themselves (driver mutations)

Question 24

without angiogensis what size can cancer cells grow to?

Answer 24

1-2mm

Question 25

what causes cancer organ tropism?

Answer 25

expression of different endothelial cells

Question 26

what is the def of metastases?

Answer 26

invasions in to the ecm

vascular dissemination