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Repro/heme Path > Heme Immunocomp > Flashcards

Heme Immunocomp Flashcards

1
Q

Chronic Granulomatous Disease

A

ploys lack NADPH oxidase
lack of oxidative burst d/t peroxide formation
pt susceptible to catalase + inf
E. coli, Aspergillus, Psuedomonas, and S.aureus are also common
infections begin first year of life
skin, lung, and soft tissue inf common

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2
Q

Myeloperoxidase Def

A

most common granulocyte disorder
lysosomal enzyme
catalyzes form of hypochlorous acid to H2O2
most ind are healthy

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3
Q

Chediak Higashi

A 
AR
defect in tubule polymerization
impaired degranulation (phagocytosis) and chemotaxis of neutrophils
partial albinism
peripheral neuropathy
susc to staph and strep inf
recurrent pyogenic inf
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4
Q

Lazy Leukocyte Syndrome

A

gingivitis
recurrent otitis media
stomatitis

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5
Q

Hyperimmunoglobulin E Syndrome (Job’s syndrome)

A 
Defective T-cell signaling --> abnormal chemotaxis --> INF-gamma def
excess IgE
cutaneous abscesses due to staph
truncated S. aureus inf present as a cold
lion facies
eczema
failure to lose baby teeth
pt often have red hair
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6
Q

Aplastic anemia

A

bone marrow failure
neutropenia
neutrophils also have impaired bactericidal activity

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7
Q

Paroxysmal Nocturnal Hemoglobinuria

A

impaired neutrophil chemotaxis

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8
Q

Sickle Cell Anemia

A

impaired neutrophil bactericidal activity

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9
Q

Etiologies of cell-mediated immune defects

A 
malig (Hodgkin's lymph, leuk)
HIV
Corticosteroids (dose and time dep)
Cytotoxic therapy
anti-rejection drugs
radiotherapy
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10
Q

Severe Combined Immunodef Syndrome

A

primary disorder of cell-med immunity (B and T cells)
defective IL2R OR def adenosine deaminase enzyme OR Bare lymphocyte syndrome (no MHC II expression)
X-linked
infants –> failure to thrive; recurrent inf
S. aureus, S. pneumoniae, H. influenzae
Pneumocystis, Candida, herpes group
usually die by 2yo

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11
Q

Wiscott-Aldrich Syndrome

A

inability to respond to polysaccharide antigen

inf d/t encapsulated organisms (s. pneumoniae and h. influenzae)

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12
Q

Ataxia-telangectasia

A

DNA repair (ATM protein) defect
cerebellar defects –> ataxia
absent IgA and sIgA
spider angiomas
pt sensitive to radiation due to inability to repair ds DNA breaks
inc risk of leukemias and lymphomas
repeated sinopulmonary infections –> encapsulated bacteria

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13
Q

DiGeorge Syndrome

A

absence of thyroid and parathyroid glands

risk of overwhelming viral and fungal infections

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14
Q

Sex-linked hypogammaglobulinemia

A

non-malignant def in humoral immunity

recurrent pyogenic inf d/t encapsulated bacteria

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15
Q

Hyperimmunoglobulin M

A

non-malignant def in humoral immunity

recurrent respiratory, soft tissue, and GI inf

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16
Q

Selective IgM def

A

non-malig def in humoral immunity

recurrent pyogenic inf

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17
Q

Selective IgA def

A

non-malig def in humoral immunity
inc URT inf
chronic diarrhea d/t Giardia

18
Q

Common variable immunodeficiency

A

non-malig def in humoral immunity
resp inf d/t h. inf, s. aureus
diarrhea d/t giardia lamblia

19
Q

C1, C2, C3 def

A

infections rare

20
Q

C3 or C5 def

A

severe inf with encapsulated bacteria
s. aureus
enteric GNR

21
Q

C5b, C6, C7, C8, C9 def

A

recurrent Neisseria inf

typically begin in teen years

22
Q

Splenectomy

A

fulminant inf due to encapsulated organisms
s. pneumoniae / h. influenzae / neisseria / capnocytophaga
pt with Sickle Cell : also Salmonella

23
Q

Pneumocystis prophylaxis

24
Q

HSV prophylaxis

25
Q

CMV prophylaxis

A

Ganciclovir

26
Q

immunization in compromised pt

A

Active: Pneumococcus, VZV
Passive: VZIG

27
Q

Accelerate granulocyte recovery

A

G-CSF

GM-CSF

28
Q

type of inf in 1st few days of neutropenia

29
Q

type of in 4-5 of neutropenia

30
Q

type of inf 1st month post-xsplant

A

bacterial

HSV

31
Q

type of inf >1mo post-xsplant

A 
HSV
VZV
CMV
Aspergillus
Nocardia
PCP
32
Q

type of inf >3mo post-xsplant

A

Cryptococcus

33
Q

Aspergillus inf in comprom pt

A

pleuritic chest pain; resembles Sx of PE

34
Q

Nodules on CT or X-ray

A

Aspergillus

Nocardia

35
Q

Interstitial infiltrate on CT or X-ray

36
Q

Focal lesions on CT or X-ray

A

bacterial, mycobacterial, or fungal inf

37
Q

Sinus involvement in comp pt

A

Fungus: Mucor or Aspergillus

38
Q

Reactive follicle with germinal center

A

tangible body macrophages

mantle zone

39
Q

myelodysplastic syndromes

A 
ineffective hematopoiesis
resulting in cytopenias
refractory anemia --> inc blast cells --> AML
slow progression
usually pt over 50
40
Q

acute leukemias

A

more curable

younger pop

41
Q

chronic leukemias

A

less curable –> not as rapidly dividing

older pop

Repro/heme Path (11 decks)

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