Session 9

Question 1

What is immunodeficiency associated with a higher risk of?

Answer 1

Autoimmune diseases

Malignancy

Question 2

Define immunocompromised host

Answer 2

State in which the immune system is
unable to respond appropriately and
effectively to infectious microorganisms

Question 3

What are the key components of the Innate immune system?

Answer 3

Innate barriers eg skin, mucus membranes
Phagocytes eg Neutrophils, Macrophages
Complement system

Question 4

What are the key componenets of the Adaptive immune system?

Answer 4

B cells
Antibodies
T Cells

Question 5

How can you recognise patients with Immunodeficiencies?

Answer 5

Infections will be - 
S  severe
P  persistent
U  unusual
R  recurrent

Question 6

What are the 2 types of Immunodeficiencies?

Answer 6

Primary

Secondary

Question 7

What is a Primary Immunodeficiency?

Answer 7

There is an intrinsic defect causing the immunocompromisation eg genetics, polymorphisms (HLA/MHC)

Question 8

What is a Secondary Immunodeficiency?

Answer 8

There is an underlying disease or condition affecting the immunity components eg Decreased production

Question 9

What are some of the presentations of patients with Primary antibody deficiencies?

Answer 9

Recurrent upper and lower respiratory
bacterial infections
GI complications
Arthropathies
Increased incidence of autoimmune disease
Increased incidence of lymphoma & gastric carcinoma

Question 10

How can you manage a patient who has Primary antibosy deficiency?

Answer 10

Prompt / prophylactic antibiotics
Immunoglobulin replacement therapy (Taken from many different people’s blood donations. Lifelong treatment)
Management of respiratory function
Avoid unnecessary exposure to radiation

Question 11

How would a patient with Phagocyte deficiency present?

Answer 11

Prolonged and recurrent infections -
• Skin and mucous membranes (ulcers)
• Commonly staphylococcal (catalase +ve)
• Invasive Aspergillosis
• Inflammatory problems (granuloma)

Question 12

How are patients with Phagocyte deficiencies managed?

Answer 12

Prophylactic antibiotics/anti-fungal agents
Steroids (CGD)
Stem cell transplantation from bone marrow match

Question 13

What is Severe Combined Immunodeficiency?

Answer 13

A primary immune deficiency. The defining characteristic is usually a severe defect in both the T- & B-lymphocytes

Question 14

How would Severe Combined Immunodeficiency present?

Answer 14

Failure to thrive
Protracted diarrhoea#
Hepato-splenomegaly
90% of SCID have low lymphocyte
count (<4.0-10.0 cells/ml)
High susceptibility to fungal and
viral infection

Question 15

How would you manage Severe Combined Immmunodeficiency syndrome?

Answer 15

Short term - No live vaccines, aggressive treatment of infections, prevention of new infections
Long term - Bone marrow/Stem cell transplant, gene therapy for future

Question 16

What causes inherited angiodema?

Answer 16

A lack of C1 inhibitor (Part of the complement system) in the system