Immunodeficiencies Flashcards
Covers basics of immunodeficiency diseases and studies primary and secondary immunodeficiencies
Immunodeficiency diseases are defective host defense systems classified as… or …
congenital (primary) or acquired (secondary)
Primary immunodeficiencies are classified in what four ways?
- B cell or humoral
- T cell or cellular
- Defects of phagocytes
- Abnormalities of the complement system
People with antibody deficiency diseases are inordinately susceptible to…
recurrent bacterial infections
Bruton X-linked Agammaglobulinemia typically appears in which sex and age?
Boys <1 year old (manifests early)
Bruton X-linked Agammaglobulinemia is characterized by a lack of… in …
lack of immature B cells in periphery and plasma
Which primary immunodeficiency is a genetic defect on long arm of X chromosome rendering B cells incapable of maturation?
Bruton X-linked Agammaglobulinemia
With Bruton X-linked Agammaglobulinemia, patients develop recurrent…
infections of mucosal surfaces
What type of diagnosis is required for Bruton X-linked Agammaglobulinemia?
Lab diagnosis
DiGeorge syndrome is caused by defective development of the third and fourth pharyngeal pouches, which give rise to… and influence…
thymic epithelium and parathyroid glands and influence conotruncal cardiac development
In the absence of functional thymus… is interrupted
T-cell maturation is interrupted at the pre-T cell stage
Which cells are effected by DiGeorge Syndrome?
T cells
Those with DiGeorge Syndrome have frequent…
recurrent infections
What is Severe Combined Immunodeficiency?
The worst of primary immunodeficiencies; encompasses over 20 disorders associated by deficiencies in T-cell and B-cell development and function
Severe Combined Immunodeficiency includes…
X-linked, autosomal recessive
Patients affected by Severe Combined Immunodeficiency present in the first few months of life with…
recurrent, often severe infections, diarrhea, and failure to thrive
What is the treatment for Severe Combined Immunodeficiency?
Stem cell graft treatment
Which primary immunodeficiency leads to spider veins?
Immunodeficiency with ataxia telangiectasia
Which primary immunodeficiency has increased susceptibility to infection, specifically frequent sinopulmonary infections?
Immunodeficiency with ataxia telangiectasia
How does immunodeficiency with ataxia telangiectasia affect DNA, T cells, and immunoglobulins?
Damages DNA by affecting repair, lowers T cell numbers as well as IgG, IgA, and IgE
Which primary immunodeficiency is an autosomal recessive disorder demonstrating increased susceptibility to infection by pyogenic bacteria?
Chediak-Higashi Syndrome
Due to defect in membrane organelles, those with Chediak-Higashi Syndrome have defective…
fusion of lysosomes and phagosomes
Patients with Chediak-Higashi Syndrome will have reduced ability to… leading to…
reduced ability to kill ingested microbes, leading to recurrent infections with pus
Which primary immunodeficiency occurs with frequent albinism of eyes and skin?
Chediak-Higashi Syndrome
What is Wiskott-Aldrich Syndrome?
X-linked disorder demonstrating increased susceptibility to infection, especially S. aureus
Which cells are affected by Wiskott-Aldrich Syndrome?
T and B cell numbers and functions reduced as well as immunoglobulins
Platelets abnormal and reduced
What would be the effect of abnormal and reduced platelets that come with Wiskott-Aldrich Syndrome?
Prolonged bleeding, easy bruising
LAD type I has deficiencies in…
Patients have…
deficiencies in integrins
Patients have localized bacterial infections which may be life-threatening
LAD type II has deficiencies in…
Patients have…
deficiencies in selectins
Patients life-threatening infections and severe developmental delay
LAD I and II are deficiencies in…
leukocyte adhesion
With LAD I and II, flow cytometry shows…
elevated WBC count with confirmation of molecular deficiency
What is physiologic sequelae?
Inability to build immune system
General malnutrition, vitamin and mineral deficiency
Ionizing radiation, chemotherapeutics, anti-inflammatory drugs, and immunosuppressants are all examples of…
therapeutic treatments for secondary immunodeficiency
Ionizing radiation damages bone marrow which will affect…
B-cells
What type of drug is prednisone?
Anti-inflammatory
What are three infectious examples of secondary immunodeficiency?
- HIV
- EBV
- Schistosoma
With HIV, what aspect of the immune system is low?
CD4+ which is important for T-cells
What happens when a patient is infected with EBV?
WBCs are transformed to cancer cells
What is schistosoma?
Parasites consume immune system which can cause cancer
Cancers like multiple myeloma, Waldenstrom’s macroglobulinemia, and leukemias and lymphomas are all examples of what type of immunodeficiency?
Secondary
What is the effect of multiple myeloma?
Destroyed bone marrow which produces immune cells
Multiple myeloma’s “lesser cousin” is…
Waldenstrom’s macroglobulinemia
Leukemias and lymphomas are all…
immune cell cancers where cancer takes over the immune system
