11. Immunodeficiency Flashcards Preview

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Flashcards in 11. Immunodeficiency Deck (30)
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1
Q

What cases should immunodeficiency be suspected in?

A
Serious, persistant, unusual and recurrent infections
Family history
HIV risk
Failure to thrive/weight loss
Lymphopaenia in infants
Reaction to live vaccines
Associated congenital malformations eg. cleft palate
Lymphoid malignancies
2
Q

What features require investigation if over 2 of them are present in an adult?

A

4+ infections requiring antibiotics in the past year
Recurring infections or require a long course of antibiotics
2+ severe bacterial infections
2+ radiologically proven pneumonia within 3 years
Infection in an unusual location or unusual pathogen
Family history

3
Q

What is physiological immunodeficiency?

A

Period of time between receiving mother’s IgG and making their own

4
Q

Name 3 primary immunodeficiencies

A

X-linked agammaglobulinaemia
CVID
Specific antibody deficiencies

5
Q

Give some examples of secondary immunodeficiencies

A

Neoplasms
Drug therapy
Splenectomy

6
Q

What antibodies are typically underproduced in CVID?

A

IgG
IgA
IgM

7
Q

What age does CVID present?

A

Any

8
Q

In what way can the lungs be damaged in CVID?

A

Scarring from recurrent infections leads to bronchiectasis

Breathing is compromised

9
Q

What does CVID increase the risk of?

A

Granulomatous disease
Auto immunity
Malignancies

10
Q

What immune defect is associated with viral, protozoal and fungal infections?

A

T cell

11
Q

What immune defect is associated with bacterial infections?

A

B cell

12
Q

What immune defect is associated with boils and abscesses?

A

Neutrophils

13
Q

What immune defect is associated with recurrent meningitis and lupus like disease?

A

Complement

14
Q

How are humoral immunity defects managed?

A

Antibiotics and vaccination
IV or subcutaneous Ig replacement
Physio and bronchodilators

15
Q

What are Howell-Jolly bodies?

A

Purple dots on RBCs that show they still have a nucleus

Property of an inactive spleen

16
Q

What type of infection is most closely associated with post splenectomy patients?

A

Encapsulated bacteria

17
Q

How are patients managed post-splenectomy?

A

Vaccination
Antibiotic prophylaxis
Advice, wear medic alert bracelet

18
Q

What are the causes of primary neutrophil dysfunction?

A

Chronic granulomatous disease
Leukocyte adhesion defect
Hyper-IgE syndrome

19
Q

What are the secondary causes of neutrophil dysfunction?

A

Steroids
Diabetes
Iron deficiency

20
Q

How do steroids cause neutrophil dysfunction?

A

Affects migration

21
Q

How does diabetes cause neutrophil dysfunction?

A

Impaired mobility due to sugar levels

22
Q

How does an iron deficiency cause neutrophil dysfunction?

A

Impaired bacterial killing as iron is needed for respiratory burst

23
Q

How is chronic granulomatous disease managed?

A

Antibiotics
Antifungals
Steroids for any inflammatory complications

24
Q

What is the cure for chronic granulomatous disease?

A

Haematopoietic stem cell transplant

25
Q

What is the difference between primary and secondary complement deficiency?

A

Primary is a genetic cause of low or dysfunctional proteins

Secondary due to the production of autoantibodies

26
Q

How is a complement deficiency managed?

A

No cure
Vaccination and prophylactic antibiotics
Patient education

27
Q

Name 3 primary T cell defects

A

SCID
ADA deficiency
CD40 ligand deficiency

28
Q

Name 4 secondary causes of T cell defects

A

HIV
Chemo and radiotherapy
Transplant recipients
Treatments for severe autoimmune diseases

29
Q

How is T cell function clinically assessed?

A
History
Lymphocyte count: T cell count and subtypes
Expression of TCRs, cytokine receptors
Delayed hypersensitivity skin tests
Vaccination with protein antigens
Proliferation assays
30
Q

How is SCID managed?

A

Irradiate all blood products to prevent GvHD
No live vaccines
Haematopoietic stem cell transplant ASAP