Immunodeficiency syndromes (complete) Flashcards
What are immunodeficiency diseases
diseases caused by defects to one or more of the components of the immune system
what are the two categories of immunodeficiency
primary and acquired
what is primary immunodeficiency
defects to immune components that are genetic
what is acquired immunodeficiency
defects to immune components that are due to infection, immunosupressive drugs or malnutrition
what are the three types of immunodeficiency
humoral
cellular
combined
what is the main problem with immunodeficiency
increased susceptibility to infection
what is usually defective in primary immunodeficiency
missing enzyme
missing cell type
non-functioning component
can primary immunodeficiency be aquired
yes
can secondary (acquired) immunodeficiency be congenital
no
what are the four types of humoral immunodeficiencies
Hypogammaglobulinemia
Agammaglobulinemia
Hyper-IgM syndrome
selective IgA deficiency
what is hypogammaglubulinemia
when you don’t have enough of a certain Immunoglobin/antibody
what is transient hypogammaglobulinemia of infancy
a normal phenomenon, in which an infant doesn’t have enough antibody/Ig. this is caused by a delay in B-cell development
how is transient hypogammalobulinemia of infancy combatted
the maternal IgG confers protection to the infant
at what point does transient hypogammaglobuilinemia of infancy resolve itself
at around 9 months, the infants develops its own Ab
What is agammaglobulinemia
no antibodies in the blood
what is bruton’s agammaglobulinemia
a deficiency in circulating mature B-cells
what causes bruton’s agammaglobulinemia’s shortage or mature B-cells
mutation/deletion of Bcell tyrosine kinase
what are the T-cells like in bruton’s agammaglobulinemia
normal
when do you start to see infectious symptoms in bruton’s agammaglobulinemia
when the mothers IgG declines
is bruton’s agammaglobulinemia primary or secondary
primary (x-linked)
is transient hypogammaglobuilinemia of infancy primary or secondary
primary
how is bruton’s agammaglobulinemia treated
- prevention of infection with vaccination (dead vaccines only)
- agressive antibiotic treatment of infections
- monthly injections of intravenous immunoglobin
What is hyper-IgM syndrome
when B-cells can’t do class switching, so you only have IgM
is hyper-IgM syndrome primary or secondary
primary (x-linked)
what are the defects that lead to hyper-IgM syndrome
- defects in CD40 on T-cell or CD40L on B-cell
2. defects in AID
what are they symptoms of hyper-IgM syndrome
predisposed to bacterial infections, fungal infection, pneumocytis jiroveci, and other opportunistic infections
how is hyper-IgM syndrome treated
monthly Intravenous immunoglobin (IVIg) injections
What is the most common immunodeficiency
IgA deficiency
what is IgA deficiency
low IgA levels
what are the symptoms of someone with IgA deficiency
normal, respiratory infections, diarrhea
what does IgA deficiency predispose someone to
- celiac disease
- oral mucosal infections
it doesn’t affect periodontal or dental health
is selective immunoglobin isotope deficiency primary or secondary
primary
what is CVID
common variable immunodeficiency, a group of disorders that lead to low Ab levels
which cell type is absent in CVID
plasma cells
how do you diagnose CVID
rule out all other types of primary immunodeficiency
is CVID primary or secondary immunodeficiency
primary
what are the levels of IgG, IgA, IgM, and b-cells in patients with CVID
low IgG, and IgA
normal/reduced IgM and B-cells
are malignant tumors common in patients with CVID
yes
What are the different types of cellular immunodeficiencies
DiGeorge syndrome
what is DiGeorge syndrome
a primary immunodeficiency where there is a defect in t-cell maturation
what are the B and T-cell levels in DiGeorge syndrome
normal B-cell levels
low or absent T-cells
what is the thymus like inDiGeorge syndrome
small
what type of infection is most common to those with DiGeorge syndrome
viral infections.
how do you treat someone with DiGeorge syndrome
transplantation with a fetal thymus
What is SCID
severe, combined immunodeficiency, a primary immunodeficiency in which both B and T-cells are very low
what are the levels of B and T-cells like in SCID
both are very low
what is the boy in the disorder that is associated with the “boy in the plastic bubble”
SCID
how does a patient get SCID
x-linked inherited
what is the treatment for someone with SCID
bone marrow transplant
what is CGD
chronic granulomatus disorder, a defect in phagocytes
what is the defect in the phagocytes that causes CGD
they can’t produce H2O2 or O2 due to a genetic defect in NADPH oxidase
what are the common infections that accompany CGD
pneumonia, lymph node infection, lung infection, abscesses on skin or liver
how is CGD treated
with long term antibiotics and IFN-y injections to activate macrophages
What can cause immunodeficiency
malnutrition, cancer, or infection (HIV)
what is acquired immunodeficiency syndrome
AIDS
what causes AIDS
HIV
how does HIV lead to immunodeficiency
it binds to CD4 on T-cells, and replicates within them. then it is processed and presented to cytotoxic T-cells, and the Cytotoxic T-cells kill off the CD4 T-cells
how do you detect HIV
anti-HIV Ab detection
how is HIV treated
triple drug therapy (HAART)
highly active anti-ertroviral therapy
what does HIV ultimately lead to
depletion of CD4+ cells = AIDS
CD4 cells are 1/10 the normal amount
what part of HIV binds to the CD4 of T-cells
gp120
can HIV lead to periodontal disease
yes (especially those with a high viral load and low CD4 T-cell counts)
what are the types of oral manifestations of HIV
aphthous ulceration oral hairy leukoplakia oral candidiasis necrotizing ulcerative periodontitis necrotizing ulcerative stomatitis (risk of PDL attachment loss and gingival recession)
what is a marker of mild immunodeficiency in the mouth, and is often the first manifestation of an HIV infected patient
oral candidiasis (white lesions on the palate)
