11 Immunodeficiency Flashcards Preview

Clinical Pathology > 11 Immunodeficiency > Flashcards

Flashcards in 11 Immunodeficiency Deck (14)
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1
Q

What is immunodeficiency?

A

Clinical situation where the immune system is not effective enough to protect the body against infection.

2
Q

Name some common causes of secondary immunodeficiency.

A

Stress, surgery, burns, malnutrition, cancer, immunosuppressive drugs, irradiation, AIDS, measles, TB.

3
Q

What causes immunodeficiency of the innate immune system? How does it present?

A

IRAK deficiency. Leads to IL1, IL6 + TNF release.

Recurrent infection at young age with CRP that isn’t elevated.

4
Q

How does IRAK deficiency change clinically over time?

A

Gets less serious as adaptive immune system develops to cover the gaps.

5
Q

How does phagocytosis work?

A

Free radical generation lowers the pH in the lysozyme, activating proteolytic enzymes which kill phagocytksed bacteria.

6
Q

Which test tests for the oxidative response seen in phagocytosis?

A

Nitroblue tetrazolium test (NBT).

7
Q

Which disease develops when phagocytosis is ineffective?
5 characteristic signs.
What is its genetic basis?

A

Chronic granulomatous disease.
Osteomyelitis, pneumonia, swollen lymph, granulomas, IBD.
X-linked gp91 mutation.

8
Q

An absent terminal complement pathway response predisposes you to which disease?

A

Meningococcal meningitis.

9
Q

What is X-linked agammaglobulinaemia? How does it present? How is it treated?

A

Loss of antibody secretion, leading to recurrent pyogenic bacterial infections (sino-pulmonary). Not diagnosed until 1-2yrs due to maternal IgG presence. Treated with antibiotics and iv IgG for life.

10
Q

Name 6 primary B cell deficiencies.

A
Common Variable Immunodeficiency. 
X-linked agammaglobulinaemia.
Autosomal recessive Hyper IgM syndrome.
IgA Deficiency.
IgG Subclass Deficiency.
Transient Hypogammaglobulinaemia of infancy
11
Q

How do Severe Combined Immunodeficiency (SCID) syndrome present? (3)

A

Recurrent opportunistic infections - commonly:
Extensive oro-phayngeal candida.
Protozoa: pneumocystitis.
Fulminant chickenpox with haemorrhagic lesions.

12
Q

What is SCID? How is it treated?

A

Defects in T cells, worse than B cell deficiencies as B cells need T cell help.
Bone marrow transplant.

13
Q

What does Adenosine Deaminase Deficiency (ADD) cause?

A

SCID.

14
Q

Name three causes of CID (less severe than CID).

A

Purine Nucleoside Phosphorylase Deficiency.
MHC Class II Deficiency.
Wiskott-Aldrich Syndrome.

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