Primary Immunodeficiency Flashcards
What are the components of Spefiic immunity
Antibodies
Cellular immunity
What is cellular immunity
Cell-mediated immunity is the activation of phagocytes, antigen-specific cytotoxic T-lymphocytes, and the release of various cytokines in response to an antigen.
What are the components of non-specific immunity
Complement
Phagocytes
What is the complement system in immunity
The complement system, also known as complement cascade, is a part of the immune system that enhances the ability of antibodies and phagocytic cells to clear microbes and damaged cells from an organism, promote inflammation, and attack the pathogen’s cell membrane.
What does primary immunodeficiency increase you chances of
tumours
infection
what is repeated infection with encapsulated bacteria a sign of
defective antibody production
What antibody deficiency leads to repeated respiratory infections and what bacteria is responsible
igG and igA
Pneumococcus or haemopgilus influenza
What does infection with staphylococci, gram nergative bacteria or fungi indicate a deficiency in
Phagocytes
What defeciency predisposes to infection from intracellular organims such as protozoa, viruses, intracellular bacteria
T cells or macrophages
T cell deficiency predisoses to infection from what
Mycobacteria
intracellular organisms such as protozoa, viruses, intracellular bacteria
What is reactivated herpes virus infection linked to?
T cell immunodeficiency
What does a defective Th17 pathway cause
Recurrent candida infection
What are 3 general causes of primary immunodeficiency
Mutations
polymorhisms
polygenic disorders
How severe is each different cause of primary immunodeficiency
Mutations - severe
polymorhisms - moderate
polygenic disorders - mild (autommunity)
What condition can many mutations cause
Severe combined immunodeficiency
Describe SCID
infants die within 2 months without treatment
stem cell transplant is cure
What is an example of a polymorphism
Human Leakcyte antigen
Mannan-binding lectin
What are common polygenic disorders
Common variable immnodeficiency (most common)
igA deficienty
Describe CVID
low levels of igG
Varible levels of a, M and B and T
causes recurrant resp tract infections
autoimmunity is common
Whats the clinical presentation of SCID
defective B and T cells and therefore develop infectinos in first few weeks of life
diarrohoea
unusual rashes
low lymphocytes count
When does CVID present
later life
What is treatment of primary immunodeficeicny
Mild - prohylactic antibiotics
Severe - immunoglobulin replacement therapy
Treatment of confirmed SCID
Avoid live vaccines stem cell transplantation gene therapy (when stem cell not available_
What is more common, primary or secondary immunodefieicny
Secondary
When does secondary immnodeficieny occur
When synthesis of key immune componenents is supressed (bone marrow infiltration) or their loss is acccelerated (kidney issues)
