Thrush - Immunodeficiency

Question 1

Primary immunodeficiencys are usually ____ or congenital; The most common Ig deficiency is ____

Answer 1

inherited; IgA

Question 2

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).

Answer 2

acquired;

Question 3

what are the major examples of primary immune deficiences

Answer 3

1. SCID (treat with bone marrow transplantation)
2. cell mediated deficiences
3. humoral deficiencies
4. phagocytic deficiencies

Question 4

what are the lymphoid deficiencies seen in primary immunodeficiency

Answer 4

1. combined immunodeficienes; affect both B and T cell
2. B cell deficiencies: pets get recurrent bacterial infections.
3. T cell deficiencies: inc infection of intracellular microbes

Question 5

In ____ deficiencies you will have a problem with phagocytosis and see increased bacterial infections.

Answer 5

myeloid

Question 6

People with SCID all have defect in ____cell compartment. You get a low number of lymphocytes circulating. Initial infections are usually fungal or ____.

Answer 6

T; viral

Question 7

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.

Answer 7

gamma; JAK; CD3

Question 8

Autosomal mutants in SCID include:

Answer 8

1. defect in RAG1/RAG2
2. defect in alpha chain of IL-2 receptor (CD25)
3. defect in adenosine deaminase (ADA) which is important in DNA synthesis

Question 9

______ 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

Answer 9

Wiskott Aldrich Syndrome (WAS)

Question 10

____ syndrome is where you have congenital thymic aplasia/hypoplasia.

Answer 10

DiGeorge

Question 11

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.

Answer 11

telangiectasia

Question 12

_____ lymphocyte syndrome is when you have a defect in MHC class _____.

Answer 12

Bare; II

Question 13

____ gene defect arises when you have lack of MHC class ____

Answer 13

TAP; I

Question 14

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.

Answer 14

hypogammagloulinemia; Ig

Question 15

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.

Answer 15

CD40/CD40L; IgA

Question 16

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.

Answer 16

NADPH

Question 17

_____ 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.

Answer 17

HIV; CD4; gp120;

Question 18

what therapies or treatments result in secondary immunodeficiency

Answer 18

1. lymphoid cancer patients
2. cancer therapy
3. organ transplant therapy
4. splenectomy

Question 19

what are some forms of therapy for immunodeficiency

Answer 19

1. passive supplementation: supplement with missing component
2. bone marrow transplant
3. genetic engineering