Thrush - Immunodeficiency Flashcards
Primary immunodeficiencys are usually ____ or congenital; The most common Ig deficiency is ____
inherited; IgA
Secondary immunodeficiencies are _____ and are deficiencies due to infections, aging, malignancies (lymphoid cancer) and therapy (to help reduce impact of autoimmune disease or treatments used to fight tissue graft rejection).
acquired;
what are the major examples of primary immune deficiences
- SCID (treat with bone marrow transplantation)
- cell mediated deficiences
- humoral deficiencies
- phagocytic deficiencies
what are the lymphoid deficiencies seen in primary immunodeficiency
- combined immunodeficienes; affect both B and T cell
- B cell deficiencies: pets get recurrent bacterial infections.
- T cell deficiencies: inc infection of intracellular microbes
In ____ deficiencies you will have a problem with phagocytosis and see increased bacterial infections.
myeloid
People with SCID all have defect in ____cell compartment. You get a low number of lymphocytes circulating. Initial infections are usually fungal or ____.
T; viral
In X linked SCID you get a defect in ____ chain of IL-2 receptor. In another type of SCID you get deficiency of ____-3 and _____ which are signaling molecules that plays an important role in activating Tcells.
gamma; JAK; CD3
Autosomal mutants in SCID include:
- defect in RAG1/RAG2
- defect in alpha chain of IL-2 receptor (CD25)
- defect in adenosine deaminase (ADA) which is important in DNA synthesis
______ is an ex of SCID and is when there is an X linked defect in protein involved in assembly of actin filaments. Severity of disease increases with age; you get a gradual loss of T and B cell function. Fatality is often due to infection or lymphoid malignancy
Wiskott Aldrich Syndrome (WAS)
____ syndrome is where you have congenital thymic aplasia/hypoplasia.
DiGeorge
Ataxia _____ is due to abnormal T cell function in which you have lack of coordination and dilation of blood vessels in the face. You get reduced T cell function and decreased Ab.
telangiectasia
_____ lymphocyte syndrome is when you have a defect in MHC class _____.
Bare; II
____ gene defect arises when you have lack of MHC class ____
TAP; I
B cell defects are seen in patients with a mutation in a signaling molecule causing Brutons _____. There is no proper _____ production in these patients due to defective B cell tyrosine kinase gene. They rearrange heavy chain but not light chain.
hypogammagloulinemia; Ig
X linked hyper IgM immunodeficiency is where individuals have a defect in ___/_____ signaling that prevents proper class switching in the B cells. You see high levels of IgM and low levels of IgG and IgA. Selective ____ deficiency is the most common.
CD40/CD40L; IgA
Chronic _____ disease is a myeloid deficiency where the phagocytic cells are not able to produce hydrogen peroxide or reactive oxygen products due to a defect in_______ oxidase activity which was confirmed by a negative result in the nitro blue tenzolium dye reductase test.
NADPH
_____ is the most famous infectious cause of secondary immunodeficiency. It infect ____ + Tcells via the viral _____ protein. Another HIV viral protein, gp41, plays a role in fusion of HIV to the host cell. In the 80’s hallmark of Aids was the appearance of Pneyocysis ureic and Kaposi sarcoma.
HIV; CD4; gp120;
what therapies or treatments result in secondary immunodeficiency
- lymphoid cancer patients
- cancer therapy
- organ transplant therapy
- splenectomy
what are some forms of therapy for immunodeficiency
- passive supplementation: supplement with missing component
- bone marrow transplant
- genetic engineering
