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MOD > Primary Adaptive Immunodeficiency > Flashcards

Primary Adaptive Immunodeficiency Flashcards

1
Q

How are Primary Adaptive Immunodeficiencies acquired?

A

Genetics

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2
Q

What are 3 Primary Adaptive Immunodeficiency - lymphocyte maturation disorders?

A

SCID
DiGeorge Syndrome
X-linked Agammaglobulinemia

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3
Q

What are 3 Primary Adaptive Immunodeficiency - lymphocyte maturation disorders

A

SCID
DiGeorge Syndrome
X-linked Agammaglobulinemia

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4
Q

What are 3 Primary Adaptive Immunodeficiency - lymphocyte activation/function disorders?

A

Hyper - IgM Syndrome
CVID
IgA Deficiency

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5
Q

What are 3 Primary Adaptive Immunodeficiency - lymphocyte activation/function disorders?

A

Hyper - IgM Syndrome
CVID
IgA Deficiency

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6
Q

SCID

A

Severe Combined Immunodeficiency

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7
Q

SCID has deficiencies in what cells?

A

B AND T cells

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8
Q

The Autosomal Recessive version of SCID lacks what and has a buildup of what?

A

Lacks Adenosine deaminase

Has a buildup of toxic purines

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9
Q

The Autosomal Recessive version of SCID lacks Adenosine Deaminase. What does that block?

A

T cell formation

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10
Q

If T cell formation is blocked, what will that suppress?

A

B cell function

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11
Q

The X-linked version of SCID has mutations in?

A

IL receptors

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12
Q

The X-linked version of SCID has mutations in IL receptors. What does this decrease?

A

T and B cells

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13
Q

SCID has deficiencies in what cells?

A

B AND T cells

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14
Q

DiGeorge Syndrome has a deletion of?

A

22q11

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15
Q

Deletion of 22q11

A

DiGeorge Syndrome

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16
Q

What cell is specifically impacted in DiGeorge Syndrome?

A

T cells due to lack of thymus

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17
Q

What pharyngeal pouches are missing/partial with DiGeorge syndrome?

A

3 and 4

- No thymus, No parathyroid, great vessel/heart abnormalities

18
Q

Symptoms of DiGeorge Syndrome?

A

T cell deficiency that could impact B cells function

- Facial/cardiac anomalies, hypocalcemia (tetany)

19
Q

Symptoms of DiGeorge Syndrome?

A

T cell deficiency that could impact B cells function

- Facial/cardiac anomalies, hypocalcemia (tetany)

20
Q

What gene on what chromosome is affected by X-linked Agammaglobulinemia?

A

BTK gene on X chromosome

Bruton Tyrosine Kinase

21
Q

Defect in BTK gene

A

X-linked Agammaglobulinemia

22
Q

What cells are affected and how with X-linked Agammaglobulinemia?

A

Pre-B cells can’t mature

23
Q

Those with X-linked Agammaglobulinemia’s risk for infection increases as?

A

Mother’s antibodies wane

24
Q

Hyper-IgM Syndrome has mutations where?

A

CD40/CD40L

25
Q

Mutations in CD40/CD40L

A

Hyper-IgM Syndrome

26
Q

What is the CD40/CD40L essential for?

A

T cells helping B cells class switch

27
Q

What mechanism is impaired with Hyper-IgM Syndrome?

A 
T cells helping B cells class switch 
-- B cells unable to class switch due to mutations in CD40/CD40L
28
Q

What antibodies will be increased with Hyper-IgM Syndrome?

A

IgM

29
Q

Will the other antibodies by increased with Hyper-IgM Syndrome?

A

NO - they will be decreased

30
Q

CVID

A

Common Variable Immunodeficiency

31
Q

What is the most common Primary Adaptive Immunodeficiency?

A

CVID

32
Q

CVID is what?

A

Multiple disorders of hypogammaglobulinemia

33
Q

CVID is a disease of_____

A

exclusion

34
Q

CVID has later presentation. What symptoms will those with CVID have?

A

Recurrent pulmonary/sinus infections
Anemia/thrombocytopenia
Chronic diarrhea

35
Q

What symptoms will those with CVID have?

A

Recurrent pulmonary/sinus infection
Anemia/thrombocytopenia
Chronic diarrhea

36
Q

IgA deficiency has decreased defense against?

A

Inhaled and Ingested pathogens

37
Q

What types of infections will those with IgA deficiency get?

A

Pulmonary
GI
Bladder

38
Q

What serious condition is associated with IgA deficiency?

A

Transfusion - related Anaphylaxis

39
Q

What is Transfusion - related Anaphylaxis?

A

Anaphylaxis with transfusion of blood that contains IgA antibodies due to those with IgA deficiency not having seen them before

40
Q

What can prevent Transfusion - related Anaphylaxis?

A

Red cell washing

MOD (68 decks)

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