Humoral Immune Deficiencies Flashcards Preview

Ch 20: Allergy and Immunology > Humoral Immune Deficiencies > Flashcards

Flashcards in Humoral Immune Deficiencies Deck (11)
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1
Q

What is the most common primary immunodeficiency?

A

IgA Deficiency

Over 80% are asymptomatic

NOT and indication for IVIG replacement

2
Q

What is Bruton’s X-linked Agammaglobulinemia (XLA)?

A

Primary affects B cells. T cells are ELEVATED

IgM, IgG, IgA, and IgE are LOW

Tonsils, Adenoids, Peyer’s Patches, Spleen, and Peripheral lymph nodes are SMALL

Get infections with encapsulated pyogenic bacteria: Strep pneumo, H. flu

At risk for bronchiectasis and chronic pulmonary insufficiency

3
Q

How do you diagnosis and treat Bruton’s X-linked Agammaglobulinemia (XLA)?

A

Diagnosis-
Measure immunoglobulin levels. When low then confirm by measuring B and T cells

Treatment-
Need IVIG

DO NOT GIVE LIVE VIRAL VACCINES

4
Q

What is Common Variable Immunodeficiency (CVD)?

A

Most common CLINICALLY SIGNIFICANT antibody deficiency.

B cells cannot differentiate into plasma cells, all Igs are deficient. T cell defects present too

5
Q

How do pts with Common Variable Immunodeficiency (CVD) present?

A

NORMAL CBCs

Recurrent infections of the upper and lower resp tract
Frequent GI sxs (chronic diarrhea and malabsorption)
Recurrent herpes and zoster infections

Increased risk for LYMPHOMA (EBV associated).
Associated with autoimmune disease (RA, cytopenias, thyroid abnormalities)

6
Q

How do you treat pts with Common Variable Immunodeficiency (CVD) present?

A

Treatment-
IVIG

DO NOT GIVE LIVE VIRAL VACCINES

7
Q

What is X-linked Hyper IgM Syndrome? How does it present?

A

Disruption of B cell differentiation due to T CELL abnormality. Can’t convert IgM to IgG.

Male 6-12 mo with frequent otitis and sinopulmonary infections, as well as diarrhea

HALLMARK: lymphoid hypertrophy despite antibody deficiency

Presence of Pneumocystis carinii without HIV infection.

8
Q

How do you treat patients with X-linked Hyper IgM Syndrome?

A

Treatment-
IVIG

DO NOT GIVE LIVE VIRAL VACCINES

9
Q

What is Job Syndrome/Hyper IgE?

A
Eosinophilia
Eczema
Elevated IgE 
*recurrent skin infections
*chronic thrush
*abnormal facies
*multiple fractures/skeletal abnormalities

Can be mistaken for atopic dermatitis, but you won’t have skeletal abn or weird facies

Different from Wiskott-Aldrich because you’ll have a milder rash and they have bleeding due to thrombocytopenia

S. aureus is the main bug!

10
Q

How do you treat Job Syndrome/Hyper IgE?

A

Treatment: antibiotics and steroids

11
Q

What is transient hypogammaglobulinemia of infancy?

A

Decreased T-helper function that leads to lower than severely LOW IgG and +/- low IgA. IgM NORMAL.

Presents by 6 mo and kids outgrow by 3-6 yo