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Haem/Onc > Immunology and Immunodeficiency, ASPHO > Flashcards

Immunology and Immunodeficiency, ASPHO Flashcards

1
Q

WBC of innate immune system?

A

neutrophils, macrophages, dendritic cells, NK cells

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2
Q

WBCs of adaptive immune system?

A

CD3/CD4 T, CD3/8 T, B cell cells

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3
Q

NK cells express?

A

CD16, 56

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4
Q

diff between CD4 and CD8 cells?

A

CD4: help B cells class switch and help CD8 T cell kill…CD8 cells have cytolytic activity

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5
Q

killing viruses requires?

A

NK cells, CD4, CD8 cells

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6
Q

killing pneumocystis, fungi requires?

A

CD4, CD8

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7
Q

killing bacteria requires?

A

b cells

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8
Q

adaptive immune system has what two arms?

A

cellular (t cells), humoral ( b cells)

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9
Q

ALC of less than ___ -____ in an infant is highly abnormal

A

1000-2000

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10
Q

how to measure quantity of lymphocytes?

A

lymphocyte subset ennumeration

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11
Q

how to measure immune function for t and b cells?

A

NONSPECIFIC:
t: proliferation to mitogens

b: measure total immunoglobulins (GAM) and can also measure IgG subclasses

SPECIFIC:

t: -proliferation to antigen for tetanus, candida
- intradermal candida control skin test

b: antigen-specfiic antibodies:
- protein: tetanus, hep b surface antigen
- carbohydrate: anti-A adn Anti-B is someone who’s blood type O; response to 23-valent pneumovax vaccine

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12
Q

3 major types of circulating immunoglob?

A

IgM, IgG, IgA

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13
Q

structure of IgM, IgG, IgA?

A

IgM: pentameric
IgG: monomeric
IgA: mono-, dimeric

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14
Q

which immunoglob has highest affinity?

A

IgG

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15
Q

which immunoglob corsses the placenta?

A

IgG…it’s small! monomeric

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16
Q

causes of immunocomproimse?

A
  • loss of physical barriers (mucosal impairment, CVCs)
  • medication (corticosteroids, cyclosporine, rituximab, etc)
  • acquired or cong defects in cell # (neutropenia, CD4 lymphopenia)
  • acquired or congen defects in cell function
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17
Q

re-do protein vaccines when after SCT?

A

~9-12 months

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18
Q

re-do live vaccines and carbohydrate vaccines when after SCT?

A

24 mos

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19
Q

alemtuzumab, ATG, methylpred (high dose) all do what to t cells?

A

lysis

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20
Q

cyclosprine and tacro do what to t-cells?

A

inhibit TCR activation and early cytokine production

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21
Q

methotrexate, mycophenolate, sirolimus do what to t cells?

A

inhibit clonal expansion, prolilferation

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22
Q

in the pre-engraftment period, day 0-30: deal with waht defect? what infections happen then?

A
  • neutropenia, catheter
  • bacterial: gram pos, neg, anaerobes
  • fungal: candida, aspergillus
  • viruses: HSV, resp
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23
Q

in the post-engraftment early period (30-100): defects? infections?

A
  • lymphopenia esp CD4
  • t cell suppressive meds
  • acute GVHD
  • catheter
    infectious: gram pos, neg, anaerobes…asperfillus…CMV, resp, EBV-Lymphoprolif disease….pneumocystitis, toxoplasma
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24
Q

if hte post-engraftment late period, >100 days: defects? infections?

A
  • lymphopenia esp CD4, t cell supp meds, cGVHD, poor Ig production
  • enapsuled organisms…aspgerillus…VZV, CMV, resp, EBV-LPD… pneumocystits, toxoplasma
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25
Q

organisms we target for F&N?

A
  • gram neg rods (e. coli, pseudomonas, klebsiella), gram pos cocci (strep viridans, staph aureus, enterococcus)
  • prevalent but mild: coag neg staph, anaerobes
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26
Q

driving risk in F&N

A

severe neutropenia, presence of catheter, mucosal barrier breaktown

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27
Q

F&N management

A

blood cultures
start antibx ASAP..dont’ wait for urine
anti-pseudomonal beta-lactams or 4th gen cepahlo or carbapenem

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28
Q

antibx ppx: give to who?

A

can consider levoflox in AML induction, relapsed ALL

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29
Q

if decide on double gram neg rod coverage, can stop that when?

A

after 48 hours of neg cultures

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30
Q

when can you stop antibx for F&N?

A
  • no fever
  • neg cultures
  • strong evidence of marrow recovery
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31
Q

fungal ppx: target what?

A 
yeast= candida
mold= aspergillus, zygomycosis, fusarium
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32
Q

who gets fungal ppx?

A
  • allo HSCT pts
  • pts undergoing AML induction/MDS
  • auto SCT recipients with neutropenia for >7 days
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33
Q

what agent should be used for fungal ppx? alternatives?

A

fluconazole

alt: echinocandin (caspo), posaconazole (at least 13 yrs), itraconazole for CGD pts

34
Q

fungal orgs ataht we target for F&N?

A

candida, aspergillus, zygomycosis, fusarium

35
Q

consider empirc fungal coverage for which F&N pts?

A

allo HSCT pts, pts undergoing AML indcution/MDS, pts with HR ALL or relapsed leukemia

36
Q

start antifungals when for F&N?

A

high risk pts with at least 96 hours of F&N that’s not responsive to broad spectrum antibx

37
Q

antifungal agent to use in F&N?

A

echinocandin (like caspo) or liposomal amphotericin b

38
Q

pneumocystitis jirovecci: who’s at risk?

A

anyone with CD4 lymphopenia (high dose chemo, HSCT, solid organ transplant, primary immunodef with low CD4 count or function

39
Q

ppx for pneumocystits jirovecii?

A

trimethoprim sulfamethoxazole 5 mg/kg/day divided BID three days per week

40
Q

3 alternatives to septra for pcp ppx?

A 
atovaquone
dapsone
inhaled pentamidine
IV pentamidine
etc
41
Q

who gets HSV ppx?

A

allo transplant who is seropositive pt <30 days

42
Q

viral ppx for HSV?

A

acyclovir(foscarnet if resistant)

43
Q

who gets CMV ppx?

A

allo transplant who is seropos themselves or who’s donor is seropositivie, give for first 100 days

44
Q

med for CMV ppx?

A

ganciclovir or valganciclovir or foscarnet or cidofovir

45
Q

who gets VZV ppx?

A

allo SCT pt who has had wild type varicella infection…give up to 1 yr post

46
Q

med for VZV ppx?

A

acyclovir IV or PO

or valacyclovir

47
Q

VZIg give when?

A

give for exposure to varicella within 96 hours

48
Q

absence of t-cells eg?

A

SCID

49
Q

present t-cells but broken eg?

A

WAS

50
Q

absence of b-cells eg?

A

XLA

51
Q

present but broken b cells eg?

A

CVID

52
Q

absence of neutrophils eg?

A

SCN

53
Q

present but broken neurphils?

A

CGD

54
Q

how does SCID get diagnosed now?

A

detected at birth via detection of ABSENT t cell receptor excision circles= ABSENT TRECs…
then check ALC, lymphocyte subsets (t cells very low or absent)
-lymphocyte proliferation studies (see absent/very low proliferation of t cells to mitogens
-total IgG, IgM, IgA
-specific Abs if vaccinated
…remember: if no t-cells, b-cells won’t work properly either!

55
Q

explanation for normal ALC but still having scid?

A

maternal engraftment

56
Q

main genes in SCID?

A

IL2RG (x-linked)= most common!!> JAK3, IL7R (both AR)

57
Q

SCID management:

A
  • protect from infection
  • IgG replacement
  • aggressive diagnosis and prompt response to infection
  • ppx against pneumocystis
  • irrad bood products!
  • if ADA deficiency, give peg-ADA
  • immediate referral to bone marrow transplant
58
Q

XLA= x-linked agammaglobulinemia: problem? tx?

A

no peripheral b cells–> becomes sick at 6-9 months once no longer has mom’s IgG; replacement IgG

59
Q

milder version of WAS?

A

x-linked thrombocytopenia: generally no eczema,normal immune function

60
Q

immune issue in WAS?

A

normal to low T clelnumber, normal B cell number but poor Ab production (carbo responses…doesn’t rspond to pneumovax)

61
Q

immune features of WAS?

A 
eczema
allergies
high ige
aiha
itp
vasculitis of the skin
arthritis
colitis
62
Q

was dx how?

A

was protein levels on flow

gene sequencing

63
Q

tx for was?

A

splenectomy
supportive
HSCt <5 yrs

64
Q

x-linked lymphoprolif disease: gene? presentations? tx?

A

SAP

  • fulminant/fatal EBV;lymphoprolif; recurretn infections with hypogamma or dysgamma
  • transplant
65
Q

CVID dx? issue?

A

decrease igg/iga/ or igm and/pr poor ab production…defects in t cell numbr or function–> autoimm, granulomatous disease, lymphoma

66
Q

infections seen in CVID?

A

sinopulmonary infections, enteroviral meningoencephalitis

67
Q

heme issues seen with CVID?

A

ITP, evans, AIHA, autoimmune neutropenia

68
Q

tx for CVID?

A

IgG replacement, supportive care for infectious and autoimmune issues

69
Q

ALPS: pathophys?

A

defect in pathways controling apoptosis of lymphocytes

70
Q

major mutation in ALPS?

A

FAS

71
Q

why are hematologists poised to see ALPS?

A

oftne prsent with autoimmune cytopenia

72
Q

what are the 2 requied criteira for ALPS?

A
  • chronic (>6 months) non malig, noninfect LAD, and/pr splenomegaly
  • elevated CD3+, TCR alpha beta + CD4 neg CD8 neg DNT cells
73
Q

accessory criteria for ALPS? (2)

A
  • defective lymphocyte apoptosis

- somatic or germline pathogenic mut

74
Q

secondary criteira for ALPS? (4)

A
  • elevated plasma sFASL or IL-10 or vit B12 or IL18
  • typical findings on histo
  • autoimmune cytoepnias and hypergammaglob
  • fam hx of nonmalig/noninfectious lymphoprolif with or without autoimmunity
75
Q

tx ALPS how?

A
  • manage cytopenias: steroids, MMF, 6MP, ritxu, sirolimus
  • role for splenectomy is controversia
  • watch for lymphoma
76
Q

immunodef indications for transplant?

A 
WAS
CD40ligand def
XLP
CGD
Leuk adhesion def
77
Q

conditoning for immunodef SCT?(not SCID)

A

busulfan (Stem cell kill) + cyclophos or fludarabine (to prevent rejection)

78
Q

why do you not need conditioning for MSD in SCID?

A

profound lack of functional t-cells…HSCs can easily engraft in the thymus adn generate t-cells

79
Q

if you have a MSD for SCID transplant do you need conditioning? t cell depletion?

A

no

no (matched t cells unlikely to cause GVHD)

80
Q

if you have a haplo parent for SCID transpalnt do you need conditioning? t cell depletion?

A

no

yes

81
Q

if you have an unrealted donor for SCID, do you need conditioning? t-cell dep?

A

yes

either one

Haem/Onc (30 decks)

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