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Primary Immunodeficiencies
Genetically Determined

B &/ or T cells affected

Increased susceptibility to infection, (esp. pyogenic/ pus)

Defect in Phagocytic activity of neutrophils or monocytes or in Chemotaxis
Secondary Immunodeficiencies
Acquired

Often Iatrogenic (medical care caused)

Infections, malnutrition, aging, side-effects deliberate immunosuppression (ie chemo & irradiation)
Predisposal due to T cell insufficiency
*** Either quantity or quality insufficiency

Mycotic & Viral infections

*** Bcells doesn't have this
Agammaglobulinemia
B-Cell Issue

X Linked

Primary Immunological Deficiency
Thymic Aplasia
T-Cell Issue

DiGeorge's Syndrome

Absent Thymus gland- decreased t-cell processing/ activity

**** viral and fungal infections
Swiss-Type Agammaglobulinemia
Both B & T

Severe lymphocytopenia

Child dies within a year- no immune system

Gene Therapy
Ataxia Telangiectasia
B & T Cell

Thymus gland is embryonic in development.

Loss of T cell & partial B cell

Loss of muscular coordination Y dilation of blood vessels
Down's Syndrome
Primary Immunological Deficiency

Increased susceptibility to infection
Monocomponent Immunodeficiencies
Lacks 'specific' component of immune system.

Defects in IgA, complement proteins & complement receptors
Lymphosarcoma, Hodgkin's Disease, multiple myeloma
Depression of serum immunoglobulins
Leukemia; myeloproliferative disorders
Depressed serum globulins
Type I: Immediate Hypersensitivity
Anaphylaxis

IgE binds to mast cell- releases histamines
Anaphylaxis
Full body edema caused by greatly increased permeability
Type II: Cytotoxic Hypersensitivity

Host cell opsonization
Antibodies attack cellular antigens

IgG & IgM

Antigen-antibody is cytotoxi or activates complement system via MAC (resulting in opsonization)
Autoimmune Hemolytic Anemia
Type II Cytotoxic Hypersensitivity

Antibodies attack own body's blood
Goodpasture's Syndrome
Type II Cytotoxic Hypersensitivity

Opsonized cells are in basement membranes of kidney and lung
Type III: Immune Complex Hypersensitivity
IgM, IgG or IgA formed against antigen

Local or systemic

More antigen than antibody
Local Immune Complex Hypersensitivity
Arthus Reaction

Takes a few hours to develop
Systemic Immune complex hypersensitivity
Serum Sickness
Serum Sickness
Classic of Systemic Type III
Type IV: Cell Mediated Hypersensitivity
Delayed T Cell responses

Hours or days after exposure

Lymphocytes

i.e. Tuberculin reaction
Organ Transplant Rejection
More involved with sensitized lymphociytes than antibodies or immune complexes