immunodeficiencies and graft rejections Flashcards Preview

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Flashcards in immunodeficiencies and graft rejections Deck (33):
1

B cell disorders

X-linked/Brutons agammaglobulinemia
selective IgA deficiency (MC)
Common variable immunodeficieny

2

X-linked/Brutons agammaglobulinemia

defect in BTK (TK gene)
no B cell maturation
X-linked recessive
recurrent bacterial and enteroviral infections >6mo
absent B cells in PBS
decrease Ig of all classes
absent lymph nodes and tonsils

3

selective IgA deficiency

defect unknown
most common primary immunodeficiency
majority asymptomatic
Airway and GI infections
Autoimmune disease
Atopy
Anaphylaxis to IgA

4

common variable immunodeficiency

defect in B cell differentiation
can be acquired in 20-30s
increased risk of autoimmune disease, brochiectasis, lymphoma, sinopulmonary infections
decreases plasma cells/Igs

5

T cell disorders

thymic aplasia/diGeorge
IL-12 R deficieny
autosomal dominant hyper-IgE syndrome (Job syndrome)
chronic mucocutaneous candidiasis

6

DiGeorge

22q11 deletion- detected by FISH
failure to develop 3rd/4th pharngeal pouches absent thymus and PT
CATCH-22

7

CATCH-22

Cardio-#1- tetralogy of fallot #2- truncus arteriosis (DiGeorge of the jungle swings into truncus of trees)
Abnormal facies
Thymic aplasia
Cleft palate
Hypocalcemia, hypoparathyroidism
22- chrom 22

8

IL-12 R deficiency

decrease Th1 response
AR
disseminated mycobacterial and fungal infections
may present after BCG vaccine
decreased IFN-Y

9

AD-hyperIgE syndrome/Jobs syndrome

deficiency of Th17 cells d/t STAT3 mutation-> imparied recruitment of neutros
FATED
increased IgE decreased IFN-y

10

FATED

course Facies
non-inflamed staph Abscesses
retained baby Teeth
increased IgE
Dermatologic (eczema)

11

chronic mucocutaneous candidiasis

T-cell dysfunction
many causes
non invasive recurretn candidasis infections
absent in vitro T cell proliferation in response to Candida
absent cutaneous rxn to candida

12

B and T cell deficiencies

SCID
Ataxia-telangectasia
Hyper-IgM syndrome
Wiskott-Aldrich syndrome

13

SCID

several types:
- defective IL-2R gamma chain (MC) X-linked
- adenosine deaminase deficiency AR
failure to thrive, chronic diarrhea, thrush, recurrent all kinds of infections
must get BM transplant

14

ataxia-telangiectasia

defects in ATM gene-> failure to repair double stranded breaks -> cell cycle arrest
triad: Ataxia, telangiectasia, IgA deficiency

15

findings for ataxia-telangiectasia

increased AFP
decreased IgA, IgG, IgE
lymphopenia, cerebellar atrophy

16

hyper IgM syndrome

MC defect is CD40L on Th cells cannot class switch
x-linked recessive
severe pyogenic infections early in life
pneumocystis, cryptosporidum, CMV
increased IgM
decreased IgG, IgA, IgE

17

Wiskott-alrich syndrome

mutation in WAS gene
x-linked recessive
T cells cannot reorganize actin cytoskelton
WATER
decreased- normal IgG, IgM
increased IgE, IgA
fewer and smaller platelets
increases risk of autoimmune diseases and malignancy

18

WATER

wiskott-aldrich
Thrombocytopenic purpura
Eczema
Recurrent infections

19

phagocyte dysfunction

leukocyte adhesion deficiency type 1
Cediak-higashi syndrome
chronic granulomatous disease

20

leukocyte adhesion deficiency type 1

defect in LFA-1 integrin (CD18) on phagocytes
autosomal recessive
recurrent bacterial sin and mucosal infections
NO pus
impaired wound healing, delayed separation of umbilical cord
increased neutros, but absent at site of infection

21

chediak-higashi syndrome

defect in lysosomal trafficking regulator gene (LYST)
microtubule dysfunction in phagosome-lysosome fusion
AR
recurrent pyogenic infections by Staph and strep
partial albinsim
peripheral neurophaty
progressive neurogeration
infiltraive lymphohistiocytosis

22

chediak-higashi fininding

giant granules in granulocytes and platelets
pancytopenia
mild coagulation

23

chronic granulomatous disease

defect of NADPH oxidase-> decreased respiratory burst in neutros
x linked MC
increased susceptibiliyt o Catalase +
abnormal dihydrorhodamine (flow cytometry) test
nitroblue tetrazolium die reduction test neg

24

Catalase +

CATs Need PLACESS
Nocardia
psuedomonas
listeria
aspergillus
candida
E. coli
S. aureus
Serratia

25

What infections do pts w/T cell deficiencies get?

Bacterial- sepsis
Viral- CMB, EBV, JVC, VZV, recurrent resp/GI
fungal/parasites- candida, PCP

26

What infections do pts w/B cell deficiencies get?

bacterial- encapsulated
viral- enteroviral encephalitis, polio (live vaccine CI)
fungal/parasites GI giardiasis (no IgA)

27

encapsulated bacteria

SHiNE SKiS
Strep pneumoniae
H. influenza
Neisseria meningitidis
E. coli
Salmonella
Klebsiella
Strep, grp B

28

What infections do pts w/granulocyte deficiencies get?

Bacterial: Staph, Burkholderia, Pseudo, Serratia, Nocarida
Fungal: Candida, aspergillus

29

what infections do pts w/decreased C'

encapsulated w/early C'
Neisseria w/MAC

30

hyperacute transplant rejection

minutes
pre-existing recipeient Abs (type II rxn)
widespread thrombosis of graft vessels -> ischemia/necrosis
graft must be removed

31

acute transplant rejection

wks-months
cellular: CD8 cells against donor MHCs
humoral: similar to hyperacute except takes time for Abs to develop
vasculitis of graft vessels w/dense interstitial lymphocytic infiltrate
prevent/reverse w/immunosuppressants

32

chronic transplant rejection

months-yrs
CD4 cell response to recipient APCs presenting donor peptides including allogenic MHC
both cellular and humoral
recipeient T cells react and secrete cytokines -> proliferation of vascular smooth mm and parenchymal fibrosis
dominated by arteriosclerosis

33

graft-vs-host disease

onset varies
grafted immnocompetent T cells proliferate in immunocomprosmises host and reject host cells w/foreign proteins -> severe organ damage
maculopapular rash, jaundice diarrhea, hepatosplenomagly, usually seen in BM and liver transplants
potentially beneficial in bone marrow transplant, graft-vs-tumor effect