Immunocompromised Host

Question 1

List the most common primary immune deficiency disease

Answer 1

Chronic viral Immunodeficiency disease (CVID)
Selective IgA deficiency 
Severe combined Immnuodeficiency (SCID)
Chronic granulomatous disease 
Severe congenital neutropenia 
Other PIDs

Question 2

Why is immunodeficiency under diagnosed?

Answer 2

Lots of phenotypes
Not well covered in medical training
Diagnostic criteria unclear

Question 3

Who gets primary immunodeficiency?

Answer 3

8-12.5 onset but most people diagnosed 18+years old which means 37% have tissue or organ damage

Question 4

What is an immunocompromised host?

Answer 4

State in which the immune system is unable to respond appropriately and effectively to an infectious organism, due to a defect in one or more accept of the immune system.

Question 5

What is the difference between primary and secondary immune deficiency?

Answer 5

Primary is congenital

Secondary is acquired

Question 6

What can the congenital immunodeficiencies mean?

Answer 6

Missing protein
Missing cell
Non-functional components

Question 7

What does the underlying disease in secondary immunodeficiency do?

Answer 7

Lowers production of immune components
Reduces the functionality of immune components
Increases degradation of immune components.

Question 8

When should you suspect immuno deficiency?

Answer 8

S- Sever
P- Persistant
U- Unusual
R- Recurrent

Any infections falling into these categories you should think of immune deficiency. Also family history of PID should ring alarm bells.

Question 9

Other than injections what are people with PID more prone to?

Answer 9

Malignancies particularly lymphoma

Autoimmune conditions

Question 10

Which PIDs cause antibody deficiency?

Answer 10

CVID

Selective IgA deficiency

Question 11

Which PID s affect T and B cells

Answer 11

Severed combined immunodeficiency (SCID)

Question 12

Name the two phagocytic defective disorders found in PID patients?

Answer 12

Chronic granulomatous disease

Severe congenital neutropenia

Question 13

What is the most common PID?

Answer 13

CVID

Question 14

What is the likely defective immune component if presentation is younger than 6 months?

Answer 14

T cells and phagocytes

Question 15

B cell/ antibody or phagocytic defects are suspected if PID onset is…..

Answer 15

between 6 months and 5 years

Question 16

If PID onset is after 5 years old what type of immunodeficiency is more likely?

Answer 16

B cell/ antibody/ complement or secondary immunodeficiency

Question 17

Complement deficiency predisposes you to what type of infection?

Answer 17

encapsulated bacterial

Question 18

A fungal infection that is either deep seated, recurrentt or invasive may make you suspect a PID, which PID type is most likely?

Answer 18

Defective phagocytes (Chronic granulomatous disease of severe congenital neutropenia)

Question 19

Antibody deficiencies may present and enteroviral infections or Giardia lamblia protazoal infections. What signs and symptoms would be seen?

Answer 19

Sinorepiratory symptoms
Arthropathies 
GI infections 
Maligancies 
Autoimmunity

Question 20

Death, failure to thrive, deep skin infections, accesses and opportunistic infections like herpes suggest which PID type?

Answer 20

T cell deficiency

Question 21

Name an X linked B cell related PID.

Answer 21

X linked Brittons disease

Question 22

How might a chance granulomatous disease patient present?

Answer 22

Pulmonary aspergillosis

Skin infections

Question 23

How do we manage PID?

Answer 23

Supportive - prevent infection, treat infections promptly, nutritional support, careful with blood products, avoid live attenuated vaccines
Specific- Immunoglobin therapy regularly and if SCID haemapoetic stem cell transplant
Co-morbidities- auntoimmunite and malignancy screening and treatment and organ damage assessments. Avoid unnecessary radiation exposure.

Question 24

What is the goal of immunoglobin therapy and who is eligible?

Answer 24

Raise serum IgG (IV or subcutaneous injections for life)

CVID, Burtons or hyper IgM syndrome patients

Question 25

What may cause a SID?

Answer 25

Malnutrition 
splenectomy 
Infections like HIV
Liver disease 
Lymphoproliferative disorders.

Question 26

Why is the spleen important in immunity?

Answer 26

We need it to combat encapsulated bacteria- Haemophillis influenzae , Strep pneumonias and neisseria meningitidis.
It makes antibodies and the splenic macrophages remove opsonised microbes and immune complexes.

Question 27

What should splenic patient do?

Answer 27

Life long prophylactic antibiotics
Immunisations against encapsulated bacteria
We are america alert bracelet or carry a card at all times

Question 28

Why are patients with a haematological cancer at increased risk of infections?

Answer 28

Chemotherapy results in neutropenia and a mucositis

Vascular catheters breach innate immunity.

Question 29

Loss or catabolism of immune components can result in SID, what kind of things lead to this?

Answer 29

Burns
Enteropathy
Nephropathy

Question 30

What lab tests are used to test for immunodeficiency ?

Answer 30

FBC
Humoral antibody levels 
Antibody response test
Neutrohil function tests 
Complement function tests

Genetic tests