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FPP MSS > Primary Immune Deficiencies + Random Shit > Flashcards

Primary Immune Deficiencies + Random Shit Flashcards

1
Q

CD31

A

PECAM1, on leukocytes & endothelial cells to help transmigration in inflammation

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2
Q

PECAM1

A

CD31, on leukocytes & endothelial cells to help transmigration in inflammation

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3
Q

vasodilation

A

histamine nitric oxide prostaglandins

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4
Q

fever

A

IL-1 TNF

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5
Q

pain

A

prostaglandins bradykinin

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6
Q

increased vascular permeability

A

histamine, serotonin C3a, C5a, bradykinin leukotrienes C4, D4, E4 platelet activating factor substance P

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7
Q

chemotaxis, leukocyte recruitment and activation

A

IL-1, TNF chemokines C3a, C5a leukotriene B4 bacterial products

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8
Q

tissue damage

A

lysosomal enzymes of leukocytes reactive oxygen species nitric oxide

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9
Q

What happens with early complement defects? (C2, C4)

A
  • sinopulmonary infections
  • increased susceptibility to S. pneumoniae, H. influenzae
  • autoimmune disease (SLE, glomerulonephritis)
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10
Q

What happens with late (C5-C9) defects?

A
  • increased susceptibility to Neisserial infections
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11
Q

What happens with C3 defects?

A
  • severe pyogenic infections
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12
Q

What lab tests should you do for complement disorders?

A
  • initial: CH50 (do this for ALL complement)
  • secondary:
    • individual component testing if CH50 is low
    • If more than 1 complement protein is low/absent –> suspect complement consumption
    • AH50 useful for rare alternative pathway defects
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13
Q

Describe the initial lab work for B-cell/antibody defects?

A

initial:

  • quantitative immunoglobulins (IgG/A/M/E)
  • vaccine titers (Diphtheria, tetanus, pneumococcus) – measures ability to make specific Abs
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14
Q

What else should you consider in B-cell/antibody defects?

A
  • CH50
  • sweat chloride (check for cystic fibrosis)
  • CT sinuses/chest
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15
Q

Describe C1 inhibitor deficiency

A
  • hereditary angioedma characterized by edema of skin and mucosal surfaces (especially periorbital)
  • (overactive C1 will overactivate complement –> vasodilation, increased vascular permeability, formation of edema)
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16
Q

Describe the secondary lab work for B-cell/antibody defects.

A

secondary:
* flow cytometry for lymphocytes subsets/B cell subsets

17
Q

What is the initial lab workup for T cell defects?

A
  • FISH (old test), RT-PCRTBX1, DNA
    chromosome microarray (preferred)
  • Lymphocyte subset enumeration (flow cytometry)
  • Numbers of T-/B-/NK-cells, memory and naïve T cells
    • ​normal infants should have almost all naive T cells, but SCID infants will show almost all memory T cells
  • CANNOT diagnose T-cell deficiency by an ALC alone
  • Immunoglobulins (quantitative, functional)
  • T-cell proliferation (Mitogens/Ag stimulation, DTH)
18
Q

Functions of IL-2R

A
  • control of peripheral self-tolerance
  • development of T-reg cells
  • differentiation of helper and cytotoxic T lymphocytes
  • in vitro expansion and differentiation of antigen-specific NK cells and T cells
19
Q

Functions of IL-4R

A
  • Regulate B-cell function in concert with IL-21
  • Immunoglobulin class switching
  • T-helper cell differentiation to Th2
  • Co-stimulant for growth in T, B, and mast cells
  • Inhibition of Th1 maturation and macrophage activation
20
Q

Functions of IL-7R

A
  • thymic development and survival of T cells
  • homeostasis of peripheral lymphocytes
  • grwoth and survival of B-cell progenitors
21
Q

Functions of IL-9R

A
  • goblet cell hyperplasia
  • mucus production
22
Q

Functions of IL-15R

A
  • Development, differentiation, survival, and activations of NK cells
  • expansion of CD8+ memory cells
  • homeostasis of peripheral T cells
23
Q

Functions of IL-21R

A
  • regulation of immunoglobulin production
  • proliferation and activation of NK cells
  • proliferation of B and T cells
24
Q

Clinical presentation of DiGeorge Syndrome (DS)?

A

CATCH22

  • Cardiac abnormalities
  • Abnormal facies
  • Thymic hypoplasia
  • Cleft palate
  • Hypocalcemia
  • Chromosome 22
25
Q

How do you diagnose DiGeorge Syndrome?

A

DNA microarray (copy number variation)

FISH

To screen: Realtime PCR of TBX1 (a homeobox protein/gene important in devo of pharyngeal pouches)

26
Q

How do you diagnose SCID?

A

Diagnostic Studies:

  • Screening Test: CBC; lymphopenia (< 1.5 k in majority of cases)
  • Confirmatory Test: Lymphocyte enumeration (T cells-naïve/memory, B cells, NK cells)
  • Newborn screening for SCID in several states (WI started in 2008)
27
Q

How do you diagnose CVID?

A
  • Decrease in IgG (>2 SD below mean) and a low IgA and/or IgM
  • IgA is low in 90%
  • if you see a low G and a low A, think CVID

Other details:

– Onset > 2yo
– Absent isohemagglutinins and poor response to vaccines
– Exclude primary Ab deficiency (XLA)
– Exclude secondary Ab deficiency
– Drugs (corticosteroids, rituximab)
– Protein losing enteropathy
– B cell lymphomas

FPP MSS (2 decks)

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