EXAM 2 IHL Diseases Flashcards Preview

IHL > EXAM 2 IHL Diseases > Flashcards

Flashcards in EXAM 2 IHL Diseases Deck (67):
1

x-linked agammaglobulinemia XLA

decrease in all Ig isotypes/ reduced b cells/ gene defective is bruton tyrosine kinase (BTK)/ no maturation beyond pre b cells/ treat by injecting with gamma globulin

2

herpes simples virus 1 HSV1 encephalitis

defective antiviral immunity/ mutation in TLR3 gene

3

Hypersensitivity type 3

IgG/ soluble antigen/ complement phagocytes/ ex: serum sickness, Arthus reaction

4

Hyper IgM syndrome

patients lack CD40L, Abnormally high serum IgM
Almost no IgG or IgA
Cannot respond to T-dependent antigens
Secondary lymphoid tissues contain no germinal centers
Aspects of cell mediated immunity are also impaired
CD40L on X chromosome, so patients with this defect are usually male/ Pneumocystis jiroveci pneumonia (PCP) presenting feature 40% cases
Neutropenia, failure to thrive, thrombocytopenia ; anemia are common
no response to protein antigens

5

AID deficiency

leads to hyper IgM syndrome

6

Hypoglycemia

antigen: Insulin receptor agonist
consequence: hypoglycemia
type II

7

X-Linked scid

cytokine receptor common gamma chain deficiency/ no IL7 leads to no maturing t cells, decreased t cells normal or increased b-cells low Ig

8

Compliment c3 deficiency

defect in compliment cascade activation/mutation in c3

9

HIV1/2

lentiviruses type of retrovirus/integrates into dna/nef molecule down regulates MHC/ kills cd4+ t cells

10

Autoimmune polyendocrine syndrome (APS1)

AIRE gene mutations severe autoimmune at young age unable to negatively select out self rec t cells in thymus becuase unable to produce self peptides

11

recurrent pyogenic bacteria infections

defective innate immune response/ mutation in MyD88 gene

12

Subacute bacterial endocarditis

Antigen: bacterial
consequence: glomerulonephritis
Type III

13

Multiple Sclerosis

antigen: myelin basic protein, proteolipid protein
Consequence: brain degeneration, paralysis

14

HPV/herpes virsus type

cytoproliferative

15

Autoimmune thrombocytopenia purpura

Antigen: platelet integrin gb IIb:IIIa
consequence: abnormal bleeding
type II

16

Type 2 diabetes

Antigen: Insulin receptor antagonist
consequence: hyperglycemia ketoacidosis
Type II

17

Type 1 diabetes

antigen: beta cell
Consequence: beta cell death
type IV

18

chediak-hidashi syndrome

defective lysosomal function in neutrophils, macrophages, dendritic cells and granule function in nk cells/ mutation in gene for lysosomal trafficking/decreased phagocytosis/ reoccurring pyrogenic infections

19

Autoimmune hemolytic anemia

Antigen: Rh blood group antigen, I antigen
consequence: destruction of RBC by compliment/phagocyte
type II

20

Hypersensitivity type 4

Th1=soluble antigen, macrophage activation, ex: contact dermatitis, tuberculin reaction
Th2= soluble antigen/ eosinophil activation, chronic asthma, chronic allergic rhinitis,
CTL=cell associated antigen, cytotoxicity, contact dermatitis

21

X-linked lymphoproliferative syndrome

uncontrolled ebv produced b cell proliferation/ defective nk cells and ctl's/ mutation in SAP

22

x-Linked hyper IgM syndrome

defects in helper t- cell dependent b and t cell activation / caused by mutation in cd40 ligand

23

DiGeorges syndrome

Decreased t cells normal b cells/ normal or decreased ig/ anomalous development of 3rd and 4th branchial pouch leads to thymic hypoplasia

24

Hypersensitivity type 2

IgG/ cell or matrix associated antigen/cell surface receptor/ mechanism: complement fcr+ cells(phogocytes nk cells), antibody alters signalling/ ex: drug allergies, chronic urticaria

25

N. Gonorrhoeae

secretes IgA protease that degrades IgA

26

Rhuematoid arthritis

antigen: synovial joint antigen
Consequence: joint inflammation and destruction
type IV/

27

autosomal recessive scid

decrease in t and b mostly t cells/ ADA and PNP deficiency accumulation of toxic metabolites in lymphoid cells

28

Suppurative/purulent

white/green puss abcess/ granulocyte infiltration

29

Gas Gangrene

necrosis of tissue bacteria Clostridia perfringens

30

Mixed essential cryoglobulinemia

antigen: Rhuematoid factor IgG complexes
consequence: systemic vasculitis
Type III

31

Graves disease

Antigen: thyroid stimulating hormone receptor, agonist (activates)
consequence: hyperthyroidism
type II

32

Ig heavy chain deficiencies

def of IgG subclasses often associated with no IgA or IgE/ chromosomal deletion at 14q32

33

Goodpastures syndrome:

Antigen:non-callagenous domain of basement membrane collegen
consequence: glomerulonephritis, pulmonary hemorrhage
type II

34

Leukocyte adhesion deficiency-2

deficient expression of leukocyte ligands for endothelial E and P selectins causing failure of leukocyte migration into tissues/ defective gene for sialyl-lewis X component of e and p selectin ligands

35

defective MHC class II (bare lymphocyte syndrome)

defective cell mediated and humoral immunity/

36

EBV (mononucleosis)

Blocks adhesion of lymphocytes to infected cells blocks icam/lfa3

37

Hashimotos

CD4 Th1 effector cells and antibodies specific for thyroid antigens, lymphocytes destroy thyroid, hypothyrodism
TYPE IV

38

Acute rheumatic fever

Antigen: strep cell wall antigen, antibodies cross react with cardiac muscle
consequence: arthritis, myocarditis, late scaring of heart valves
Type II

39

GVHD

grafted t cells to host acute<100 days / chronic>100 days
treat with immunosupression

40

Hypersensitivity type 1

Immune Reactant: IgE /soluble antigen/ mast cell activation/ EX: allergic rhinitis, asthma, systemic anaphylaxis, immediate hypersensitivity

41

Gene responsible for Burkitts Lymphoma

MYC protooncogene

42

Systemic Lupus erythematosus

Antigen: DNA, Histones, RNA, snRNP
consequence: glomerulonephritis, vasculitis, arthritis
type III

43

TB endemic fungi can lead to....

Mononuclear granulomas

44

SCID

RAG 1 and Rag2 deficiency treatment bone marrow transplant

45

compliment c2 and c4 deficiency

no classical compliment activation failure to clear immune complexes lupis like disease

46

pemphigus vulgaris

Antigen: epidermal cadherin
consequence: blistering of the skin
type II

47

Leukocyte adhesion deficiency-1

deficient expression of Beta2 integrins causing no adhesion of leukocytes/ defective Beta2 gene

48

Myasthenia Gravis

Antigen: Acetylcholine receptor antagonists (blocking)
consequence: progressive weakness

49

Chronic granulomatous disease

defective production of reactive oxygen intermediates by phagocytes/mutation in phagocyte oxidase enzyme like b558

50

beta 2 microglobulin chain deficiency

no mhc class I= ctl deficiency

51

Influenza virus

H1N1 H=hemagluttinin N=nueraminidase

52

cyclosporin

blocks t cell cytokine expression suppress immune inhibit calcineurin

53

rapamycine

blocks mtor il 2 signalling

54

block IL-6

RA less inflammation

55

adalimumab

binds tnf alpha RA, psorisis

56

Rutuximab

binds cd 20 acts to have nk cells kill b cells treatment for RA and non hodkins lypmoma

57

Gardisil/cervarix

prophylactic against HPV

58

provenge

treatment for metastatic prostate cancer

59

apeced

incomplete deletion of self reactive t cells

60

HCMV

interferes with class 1 MHC

61

mycrobacterium tuberculosis avoid death by

prevention of binding of lysosoomal and phagosome

62

lysteria monocytogenes

hides in sytosol

63

IBD

lack of t-regs

64

foxp3

ipex

65

gonherrhea

varies surface atigens

66

ALPS

Fas ligand def

67

bare lyphocyte syndrome

only missing mhc 2