Immunology: Primary Immune Deficiencies Flashcards
Primary Immune Deficiencies:
What are the CD4, CD8, B Cell, IgM and IgG changes in:
- SCID
- Di George
- BLS

Primary Immune Deficiencies:
What are the CD4, CD8, B Cell, IgM, IgG and IgA changes in:
- SCID
- Brutons
- Hyper IgM
- Selective IgA Deficiency
- CVID

Primary Immune Deficiencies:
What are the Neutrophil count, Leukocyte adhesion markers, Nitroblue test of oxidative killing (NBT) and pus findings in:
- Kostmann syndrome
- Leukocyte adhesion deficiency
- Chronic Granulomatous disease

Primary Immune Deficiencies:
What is are the subclasses of Primary immune deficiencies?
“BACKLIT CHAMP R”
- B Lymphocyte Maturation
- Alternative pathway
- Classical pathway
- Killing
- Lymphoid progenitors
- Immune barriers
- Terminal common pathway/T Cell maturation/T Cell acitvation
- C3
- HAem stem cells
- Mannose binding lectin/Mobilisation of Phagocytes
- Phagocytosis
- Recruitment of other cells
Primary Immune Deficiencies:
What are the features, disease and disease characteristics of External epithelia deficiencies?

Primary Immune Deficiencies:
What are the features, disease and disease characteristics of Mucosal surfaces deficiencies?

Primary Immune Deficiencies:
What are the features, disease and disease characteristics of Commensal bacteria deficiencies?

Primary Immune Deficiencies:
What are the features and disease characteristics of Reticular dysgenesis?

Primary Immune Deficiencies:
What are the features and disease characteristics of Kostmann syndrome?

Primary Immune Deficiencies:
What are the features and disease characteristics of Cyclic neutropenia?

Primary Immune Deficiencies:
What are the features and disease characteristics of Leukocyte adhesion deficiency?

Primary Immune Deficiencies:
What are the features, disease and disease characteristics of Phagocytosis deficiencies?

Primary Immune Deficiencies:
What are the features and disease characteristics of Chronic granulomatous disease? Including:
- Absent respiratory burst
- Excessive inflammation

Primary Immune Deficiencies:
What are the features and disease characteristics of Chronic granulomatous disease? Including:
- Symptoms
- Investigations performed

Primary Immune Deficiencies:
What are the features, disease and disease characteristics of Recruitment of other cell deficiencies?

Primary Immune Deficiencies:
What are the features, disease and disease characteristics of Alternative Pathway deficiencies?

Primary Immune Deficiencies:
What are the features and disease characteristics of Deficiencies in early classical Pathway (C1/2/4)?
Include:
- Aetiolgy
- Depoisition sites

Primary Immune Deficiencies:
What are the features and disease characteristics of Deficiencies in early classical Pathway (C1/2/4)?
Include:
- Complement deficiency
- Clinical phenotype

Primary Immune Deficiencies:
What are the features and disease characteristics of Secondary Classical Pathway deficiencies?

Primary Immune Deficiencies:
What are the features, disease and disease characteristics of Mannose binding lectin deficiencies?

Primary Immune Deficiencies:
What are the features and disease characteristics of C3 Deficiencies?

Primary Immune Deficiencies:
What are the features and disease characteristics of Secondary C3 deficiencies?
Don’t confuse with secondary Classical pathway deficiencies, which are caused by active lupus depleting the complement

Primary Immune Deficiencies:
What are the features, disease and disease characteristics of terminal common pathway deficiencies?

Primary Immune Deficiencies:
What are the features, disease and disease characteristics of Haem stem cell deficiencies?

Primary Immune Deficiencies:
What are the features and disease characteristics of SCID?
Include:
- Clinical presentation
- Family history

Primary Immune Deficiencies:
What are the features and disease characteristics of SCID?
Include:
- Associated Pathways
- Effect on Lymphocytes

Primary Immune Deficiencies:
What are the features and disease characteristics of X-linked SCID? Include:
- Epidemiology
- Genetics

Primary Immune Deficiencies:
What are the features and disease characteristics of X-linked SCID? Include:
- Phenotype

Primary Immune Deficiencies:
What are the features and disease characteristics of DiGeorge syndrome?

Primary Immune Deficiencies:
What is the clinical presentation of Bare lymphocyte Type II syndrome?

Primary Immune Deficiencies:
What is the aetiology of Bare lymphocyte Type II syndrome?

Primary Immune Deficiencies:
What are the features and disease characteristics of Bruton’s X-linked hypogammaglobulinemia?

Primary Immune Deficiencies:
What are the features and disease characteristics of Hyper IgM Syndrome? Include:
- Definition
- Genetics

Primary Immune Deficiencies:
What are the features and disease characteristics of Hyper IgM Syndrome? Include:
- Symptoms
- Complications

Primary Immune Deficiencies:
What are the features and disease characteristics of Common variable immune deficiency?

Primary Immune Deficiencies:
What is are the subclasses of Primary immune deficiencies affecting Immune barriers?
Immune barriers:
- External epithelia
- Mucosal surfaces
- Commmensal Bacteria
Primary Immune Deficiencies:
What is are the subclasses of Primary immune deficiencies affecting:
- Mobilisation of Pagocytes
- T Cell activation and effector functions
- T Cell maturation
- B Lymphocyte Maturation
Mobilisation of Phagocytes:
- Production of neutrophils
- Neutrophil maturation
- Migration to site of infection
T Cell activation and effector functions:
- Cytokine release
- T/B Cell communication
T Cell Maturation:
- Thymus
- Positive and negative selection
- Exposure of mature T cells to periphery
B Lymphocyte Maturation:
- Pro B Cells
- Class switching