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First Aid Allergy and Immunology > First Aid, Chapter 8, Immunologic Disorders, Antibody Deficiencies > Flashcards

First Aid, Chapter 8, Immunologic Disorders, Antibody Deficiencies Flashcards

1
Q

When should antibody deficiencies be considered?

A

Recurrent sinopulmonary infections

Enteroviral infections (XLA)

Giardiasis

Autoimmune phenomenon

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2
Q

In selective IgA deficiency, what are CD19/20, IgG, IgA, IgM levels and postimmunization levels?

A

CD 19/20 Normal

IgG Normal

IgA

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3
Q

In XLA, what are CD19/20, IgG, IgA, IgM levels and postimmunization levels?

A

CD 19/20 Very low/absent

IgG

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4
Q

In CVID, what are CD19/20, IgG, IgA, IgM levels and postimmunization levels?

A

CD 19/20 Normal or low

IgG

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5
Q

In Hyper IgM, what are CD19/20, IgG, IgA, IgM levels and postimmunization levels?

A

CD 19/20 Normal

IgG

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6
Q

What are the infections associated with transient hypogammaglobinemia of infancy (THI)?

A

Sinopulmonary GI, thrush, meningitis (usually not severe) May be asymptomatic

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7
Q

What are the lab findings in transient hypogammaglobinemia of infancy?

A

IgG

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8
Q

What is the therapy of transient hypogammaglobinemia of infancy?

A

Mostly no Rx, may need prophylactic antibiotics, but rarely IVIG Usually resolved by 2–4 years old

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9
Q

What is the most common primary immunodeficiency disorder?

A

Selective IgA deficiency

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10
Q

What is the inheritance of selective IgA deficiency?

A

Unknown Some sIgAD + CVID has TACI mutation Most common primary immunodeficie ncy disorder

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11
Q

What are the infections in selective IgA deficiency?

A

Majority asymptomatic; Sinopulmonary , GI infections;

Autoimmune and atopic disease;

Rare: anti-IgA Ab -> transfusion reactions

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12
Q

What are the lab findings in selective IgA deficiency?

A

IgA

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13
Q

What are causes of secondary IgA deficiency?

A

Antiepileptics Sulphasalazin ecaptopril, thyroxin

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14
Q

What conditions might IgA deficiency be a part of?

A

Ataxia telangectasia

IgG2 subclass deficiency

chronic mucocutaneous candidiasis

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15
Q

What is the treatment for selective IgA deficiency?

A

Treatment and prophylactic antibiotics; and IVIG if concomitant specific antibody defect Monitor progression to CVID

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16
Q

What are the infections and clinical findings in specific antibody deficiency?

A

Sinopulmonary infections Allergic rhinitis

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17
Q

What are the lab findings in specific antibody deficiency?

A

Normal IgG, A, M Poor polysaccharide response despite pneumovax

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18
Q

What age does specific antibody deficiency occur in?

A

> age 2

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19
Q

What is the treatment for specific antibody deficiency?

A

Prophylactic ABx and IVIG Monitor progression to CVID

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20
Q

What is the mutation of X linked aggamaglobinemia?

A

BTK (Bruton’s tyrosine kinase)

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21
Q

What are the infections in XLA?

A

Sinopulmonary infections, atypical bacteria, GI infections, enteroviral encephalitis, septic arthritis, lymphoreticular and colorectal malignancies, bronchiectasis

Small/absent lymphoid tissue, no germinal cente

22
Q

What are the lab findings in XLA?

A

Low IgG, A, M (

23
Q

What stage does the maturation of B cells arrest at in XLA?

A

Maturational arrest at the pre-B lymphocyte stage

24
Q

What types of test should you avoid to test for infectious disease in XLA?

A

Do not use serologic assays to diagnose infectious diseases—ex. ELISA for HIV Use PCR assays or cultures instead

25
Q

What is the treatment for XLA?

A

IVIG treatment and ABx Rx

26
Q

What is contraindicated in XLA?

A

Live immunizations contraindicated

27
Q

What is the inheritance/mutation in autosomal recessive agamma-globulinemia?

A

Surrogate light chain (V pre-B; λ5), μ IgM heavy chain (Cμ), Igα, Igβ, BLNK

28
Q

What are the infections/clinical findings in autosomal recessive agammaglobulinemia?

A

Same as XLA

Can be more severe and earlier onset than XLA

(XLA - Sinopulmonary infections, atypical bacteria, GI infections, enteroviral encephalitis, septic arthritis, lymphoreticula r and colorectal malignancies, bronchiectasis Small/absent lymphoid tissue, no germinal center)

29
Q

What are the lab findings in autosomal recessive agammaglobulinemia?

A

IgG, A, M (

30
Q

What is the most common mutation in autosomal recessive agammaglobulinemia?

A

μ IgM heavy chain—most common of AR agammaglobulinemia

31
Q

What is the treatment for autosomal recessive agammaglobulinemia?

A

Same as XLA, which is:

IVIG treatment and ABx Rx Live immunizations contraindicated

32
Q

What are the mutations in CVID?

A

Mostly unknown; ICOS, TACI; BAFF-R, CD19 complex, CD20

33
Q

What are the infections/clinical findings in CVID?

A

Age >2 years old, sinopulmonary, GI infections, meningitis

Bronchiectasis, BOOP, autoimmune disease, GI/liver disease, granulomatous disease, nonHodgkin’s lymphoma, and gastric carcinoma

34
Q

What are the lab findings in CVID?

A

IgG (

35
Q

What is Good’s syndrome?

A

CVID + thymoma

36
Q

What is associated with the noninfectious complications of CVID?

A

Reduced # of switched memory B cells shown to be associated with certain noninfectious complications (hematologic autoimmunity)

37
Q

What is the treatment for CVID?

A

IVIG treatment and ABx Rx

No live vaccine

Pulmonary hygiene for bronchiectasis

Excise thymoma if present

38
Q

What are the infections/clinical findings in IgG subclass deficiency?

A

mostly asymptomatic

39
Q

What are the lab findings in IgG subclass deficiency?

A

Normal IgG and low level of ≥1 subclasses

IgG2 deficiency can occur with SIgAD with impaired Ab response to polysaccharide

40
Q

Is IgG sublcass deficiency a true primary immunodeficiency?

A

Controversial if true PIDD—20% of population have subnormal of ≥1 subclasses (esp. IgG4)

41
Q

What is the treatment for IgG subclass deficiency?

A

Rx as SAD in case of poor polysaccharide response

42
Q

What is the inheritance/mutation in hyper IgM 2?

A

AR

AID deficiency

43
Q

What is the inheritance/mutation in hyper IgM 4?

A

AR

UNG deficiency

44
Q

What are the infections and clinical findings in hyper IgM2 and hyper IgM4?

A

Sinopulmonary and GI infections;

Lymphoid hyperplasia and adenopathy

CVID-like, but have increased autoimmune disease

45
Q

What are the lab findings in Hyper IgM 2 and hyper IgM 4?

A

↓IgG, IgA, IgE, and normal or ↑ IgM;

Normal T-cell function

↑LN and giant germinal centers.

46
Q

What are the AID and UNG genes required for?

A

AID (activation-induced cytidine deaminase) and UNG (uracil-DNA glycosylane) are required for class switch recombination and somatic hypermutation of B cell

47
Q

Why are Hyper IgM2 and 4 less severe than Hyper IgM1, 3, and NEMO?

A

No T-cell defect -> less severe than HIGM1, 3 and NEMO defec

48
Q

What is the therapy for hyper IgM 2 and 4?

A

IVIG

ABx Rx

No live vaccines

49
Q

What stage of B-cell development is affected by BTK mutation?

A

Arrest in pre-B–cell stage

50
Q

What are the differences between HIGM1/3 and HIGM2/4?

A

HIGM1/3 are combined immune deficiencies with more severe/wide spectrum of infections with absent LN and germinal centers due to defects in CD40L-CD40 interactions. HIGM2/4 are antibody deficiencies with less severe infections. Lymphoid hyperplasia and adenopathy are noted. Defects are in Bcell class switching and somatic hypermutation

First Aid Allergy and Immunology (68 decks)

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