Immunodeficiency Syndrome Flashcards
Primary Immunodeficiency
genetic defects, rare
Acquired Immunodeficiency
secondary due to infection, immunosuppressive drugs or malnutrition
types of acquired immunodeficiency
humoral(plasma), cellular, combined
main consequence of immunodeficiency
increased susceptibility to infection
Primary Immunodeficiency
intrinsic defects (3)
- Missing enzyme
- missing cell type
- Non-functining component
Congenital/Aquired
Secondary Immunodeficiency
Underyling disease (4)
- Lymphoid malignancy
- Infection (HIV)
- Malnutrition
- Immunosuppressive drugs
always acquired
Primary/Congenital Humoral Immunodeficiencies
- Hypogammaglobulinemias (low leve Ab)
- Agammaglobulinemias (No Ab)
- Hyper-IgM syndrome (only IgM, no class switch)
- Selective IgA deficiency (mucosal infection)
Transient Hypogammaglobulinemia of Infancy (Primary)
- Normal, physiological phenomenon
- delay in B-lymphocyte development results in decreased Ab levels
- usually doesn’t require therapy
Symtoms
- increased frequency of bacterial infections
Infant’s maternal IgG
- from collustrum
- lasts for about 1 month
at 9 months an infant
develops own Ab
Bruton’s Agammaglobulinemia
(X-linked, Primary)
trigger
treatment
Trigger
- circulating mature B cell deficiency
- mutation/deletion of B cell tyrosine kinase (Btk) gene
- Failure of B cells to mature in bone marrow
- Absence of Ab in the blood
- T cells normal, B cell issue
Treatment
- prevent infections via vaccinations
- antibiotics
- monthly injections of intravenous immunoglobulin (IVIg)
Hyper-IgM Syndrome
(X-linked, Primary)
Trigger
symptoms
treatment
Trigger
- B lymphocytes can’t class switch (only IgM)
- defects/muations in CD40L
- or defects in activation-induced (AID) enzyme
- Th cells do not stimulate B cells to undergo heavy chain isotype switching (IgM)
- lack of IgG
Symptoms
- easily gets infections ( bacterial, fungal, opportunisitic)
Treatment
- monthly injection of intravenous immunoglobulin (IVIg)
Selective Immunoglobulin Isotype Deficiency (Primary)
trigger
symtoms
Effects on oral health is unclear
Symtoms
- respirtory infections and diarrhea
- Celiac disease
- oral mucosal infections (Herpes…)
Trigger
- IgA deficiency
- most common immunodeficiency in caucasians
- Low serum IgA
Common Variable Immunodeficiency (CVID)
triggers
treatment
- Most common Antibody deficiency
- Defects in B cell differentiation
- Mature B cells are present
- No plasma cells
- Patients have low levels of IgG and IgA
- causes infections
- more malignant tumors
- shortened lifespan
Treatment: receive IVIg
DiGeorge Syndrome
- Cellular Immunodeficiencies (primary)
- Defect in T lymphocytes (thymus doesn’t form)
- Normal B lymphocytes
- Recurrent viral infections
Treatment
- transplantation with a fetal thymus
