Immunocompromised Host Flashcards
List the most common primary immune deficiency disease
Chronic viral Immunodeficiency disease (CVID) Selective IgA deficiency Severe combined Immnuodeficiency (SCID) Chronic granulomatous disease Severe congenital neutropenia Other PIDs
Why is immunodeficiency under diagnosed?
Lots of phenotypes
Not well covered in medical training
Diagnostic criteria unclear
Who gets primary immunodeficiency?
8-12.5 onset but most people diagnosed 18+years old which means 37% have tissue or organ damage
What is an immunocompromised host?
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.
What is the difference between primary and secondary immune deficiency?
Primary is congenital
Secondary is acquired
What can the congenital immunodeficiencies mean?
Missing protein
Missing cell
Non-functional components
What does the underlying disease in secondary immunodeficiency do?
Lowers production of immune components
Reduces the functionality of immune components
Increases degradation of immune components.
When should you suspect immuno deficiency?
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.
Other than injections what are people with PID more prone to?
Malignancies particularly lymphoma
Autoimmune conditions
Which PIDs cause antibody deficiency?
CVID
Selective IgA deficiency
Which PID s affect T and B cells
Severed combined immunodeficiency (SCID)
Name the two phagocytic defective disorders found in PID patients?
Chronic granulomatous disease
Severe congenital neutropenia
What is the most common PID?
CVID
What is the likely defective immune component if presentation is younger than 6 months?
T cells and phagocytes
B cell/ antibody or phagocytic defects are suspected if PID onset is…..
between 6 months and 5 years
If PID onset is after 5 years old what type of immunodeficiency is more likely?
B cell/ antibody/ complement or secondary immunodeficiency
Complement deficiency predisposes you to what type of infection?
encapsulated bacterial
A fungal infection that is either deep seated, recurrentt or invasive may make you suspect a PID, which PID type is most likely?
Defective phagocytes (Chronic granulomatous disease of severe congenital neutropenia)
Antibody deficiencies may present and enteroviral infections or Giardia lamblia protazoal infections. What signs and symptoms would be seen?
Sinorepiratory symptoms Arthropathies GI infections Maligancies Autoimmunity
Death, failure to thrive, deep skin infections, accesses and opportunistic infections like herpes suggest which PID type?
T cell deficiency
Name an X linked B cell related PID.
X linked Brittons disease
How might a chance granulomatous disease patient present?
Pulmonary aspergillosis
Skin infections
How do we manage PID?
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.
What is the goal of immunoglobin therapy and who is eligible?
Raise serum IgG (IV or subcutaneous injections for life)
CVID, Burtons or hyper IgM syndrome patients
What may cause a SID?
Malnutrition splenectomy Infections like HIV Liver disease Lymphoproliferative disorders.
Why is the spleen important in immunity?
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.
What should splenic patient do?
Life long prophylactic antibiotics
Immunisations against encapsulated bacteria
We are america alert bracelet or carry a card at all times
Why are patients with a haematological cancer at increased risk of infections?
Chemotherapy results in neutropenia and a mucositis
Vascular catheters breach innate immunity.
Loss or catabolism of immune components can result in SID, what kind of things lead to this?
Burns
Enteropathy
Nephropathy
What lab tests are used to test for immunodeficiency ?
FBC Humoral antibody levels Antibody response test Neutrohil function tests Complement function tests
Genetic tests
