Immunodeficiencies Flashcards
What causes primary immune deficiencies?
Single genetic disorders
What are the warning signs of primary immune deficiencies?
Severe Persistent Unusual Recurrent Family history
What are the types of primary immune deficiency?
Antibody deficiencies
Cellular immunodeficiencies
Innate immune disorders
What causes antibody deficiencies?
Defective B cell function or absence of mature B cells
What are the common symptoms of antibody deficiencies?
Recurrent bacterial infections of respiratory tract
What are the common infective organisms in antibody deficiencies?
S. pneumoniae
H. influenzae
What causes cellular immunodeficiencies?
Impaired T cell function or absence of normal T cells
What are the clinical features of cellular immunodeficiencies?
Unusual or opportunistic infections
Failure to thrive
What is 22q11 syndrome?
Microdeletion syndrome causing cellular immunodeficiencies
What are the common clinical features of 22q11?
Congenital cardiac anomalies Palatal deefcts Immunodeficiency Learning difficulties Characteristic face
What are the immune issues in 22q11?
Thymus a-/hypo-plasia
Recurrnent RTIs
Autoimmune conditions in 30%
What is the management of 22q11 immune issues?
Thymus transplant
What are the causes of innate immune disorders?
Defects in phagocyte function
Complement deficiencies
Absence or polymorphisms in pathogen recognition receptors
What are the clinical features in defects in phagocyte function?
Sepsis
Skin lesions
Abscesses
Lung, brain or bone infections
What are the common causative organisms in phagocyte dysfunction?
S. aureus
Aspergillus infection
What is the common causative organism in complement deficiencies?
N. meningitides
What is hereditary angioedema?
C1 inhibitor deficiency
What are the clinical features of chereditary angiodema?
Recurrent episodes of painless, non pitting, non erythematous swelling of subcutaneous tissues, intestines, oropharynx
What is the management of hereditary angioedema?
Emergency- laryngeal/oharyngeal obstruction, acute abdominal pain
C1 inhibitor infusion or FFP
What is the general management of primary immune deficiencies?
Symptomatic
Prophylaxis
Genetic counselling and prenatal diagnosis
Causative treatment
What are some options for causative treatment in primary immunodeficiency?
Ig substitution
Gene therapy
Stem cel tranplant
Thymus transplant
What are secondary immune deficiencies?
Acquired diseases affecting immune system and/or treatment negatively influencing immune system
What are the causes of secondary immune deficiency?
Environmental
Iatrogenic
Disease
What are some environmental causes of secondary immune deficiency?
Malnutrition
Trauma
Burns
What are some iatrogenic causes of secondary immune deficiencies?
Splenectomy
Drugs- immunosuppressive, anti-rheumatic, anti-epileptic
What are some diseases that can cause secondary immune deficiencies?
Infection
DM
Renal failure
Malignancy
