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SFM Quiz 2 > Immunodeficiencies > Flashcards

Immunodeficiencies Flashcards

1
Q

What are the SCID immunodeficiencies

A
  1. ADA
  2. PNP
  3. Artemis
  4. RAG1/RAG2
  5. Jak3
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2
Q

What are the selective B cell immunodeficiencies?

A
  1. BTK-agamma-globulinema
  2. Agamma-globulinemia
  3. IgA
  4. IgG subclass
  5. Hyper-IgM Syndrome
  6. Transient Hyper IgM
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3
Q

What are the selective T cell immunodeficiencies?

A
  1. Common gamma chain
  2. IL-7R alpha chain
  3. BLS-II
  4. MHC CLass I
  5. CD3
  6. DiGeorge Syndrome
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4
Q

ADA

A 
T- B- NK-
IgM- IgG- IgA-
Autosomal recessive
Severe opportunistic infections
No live vaccines
HSCT
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5
Q

PNP

A 
T- B- NK-/+
Often normal Ab panel
Autosomal recessive
Severe opportunistic infections
No live vaccines
HSCT
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6
Q

Artemis

A 
T- B- NK+
Ab panel: Some mutations, may be low 
Autosomal recessive radiosensitive
Severe opportunistic infections
No live vaccines
HSCT
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7
Q

RAG1/RAG2

A 
T- B- NK+
IgM- IgG- IgA-
Autosomal recessive
Severe opportunistic infections
No live vaccines
HSCT
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8
Q

Jak3

A 
T- B+ NK-
IgM+ IgG- IgA-
Autosomal recessive
Severe opportunistic infections
No live vaccines HSCT
*Causes defect in IL-2 receptor signaling
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9
Q

What type of inheritance do all SCIDs have?

A

Autosomal

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10
Q

Which SCID deficiency causes defective expression of pre-TCR and pre-BCR?

A

RAG1/RAG2 defeciency

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11
Q

BTK agammaglobulinemia

A 
T+ B- NK+
IgM- IgG- IgM-
X linked
Recurrent bacterial infections
No live vaccines 
HSCT
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12
Q

Agammaglobulinemia

A 
T+ B- NK+ 
IgM- IgG- IgA-
Autosomal
Recurrent bacterial infections
No live vaccines
HSCT
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13
Q

IgA

A 
T+ B+ NK+
IgM+ IgG+ IgA-
Autosomal- higher in males
Mostly asymptomatic
No restrictions
Symptomatic tx
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14
Q

IgG Subclass

A 
T+ B+ NK+
IgM+ IgG+ IgA+
Autosomal
Mostly asymptomatic
No restrictions
Symptomatic
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15
Q

Hyper IgM Syndrome

A 
T+ B+ NK+
IgM+ IgG- IgA-
X-linked & Autosomal
Encapsulated opportunistic infections
POLIO not recommended
Symptomatic tx
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16
Q

Transient Hyper IgM

A 
T+ B-/+ NK+
IgM+ IgG- IgA-
N/A 
Encapsulated opportunistic infections
POLIO no recommended
Symptomatic tx
17
Q

Common gamma chain

A 
T- B+ NK-
IgM+ IgG- IgA-
X-linked
Severe opportunistic infections
No live vaccines
HSCT
18
Q

IL-7R alpha chain

A 
T- B+ NK+
IgM+ IgG- IgA-
Autosomal recessive
Severe opportunistic infections
No live vaccines
HSCT
19
Q

BLS II

A 
CD4 lymphepenia
hypogammaglobulinemia
Autosomal recessive
Severe opportunistic infection
No live vaccines
HSCT
20
Q

MHC Class I

A 
CD8 low functional NK deficiency
IgM+ IgG+ IgA+
Autosomal recessive
Recurrent viral infections
No restrictions
Symptomatic tx
21
Q

CD3

A 
T- B+ NK+
IgM+ IgG- IgA-
AUtosomal recessive
Recurrent viral infections
No live vaccines
HSCT
22
Q

DiGeorge Syndrome

A 
T- B+ NK+
Usually normal Ab panel
Autosomal dominant
Recurrent viral infections
No restrictions

SFM Quiz 2 (10 decks)

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