Immuno Def. Deck Flashcards Preview

BPM 3 > Immuno Def. Deck > Flashcards

Flashcards in Immuno Def. Deck Deck (20)
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1
Q

X-linked hyper-IgM Symdrome

A

Function Def: Defect in T-cell dependent B cell and macrophage activation

Mech of Def. Mutation in CD40 ligand

2
Q

Common Variable Immunodeficiency

A

Function Def: Reduced or non production of selective isotypes , susceptibility to bacterial infection

Mech of Def. Mutations in receptors for B cell growth factors, costimulators

3
Q

Bare Lymphocyte Syndrome

A

Function Def:Lack of Class II MHC expression, impaired CD4 T cell activation, defective cell mediated immunity.

Mech of Def. Mutation in genes encoding transcription factor required for class II MHC gene expression

4
Q

Defect in T cell receptor complex expression or singaling

A

Function Def: Decreased T cells or abnormal ratio of CD4 and CD8, decreased cell mediated immunity

Mech of Def. Rare, due to mutations or deletions in genes encoding CD3 proteins

5
Q

Defects in Th1 diff.

A

Function Def: Decreased T cell mediated macrophage activation, susceptibility to infection

Mech of Def. Rare, due to mutations encoding the receptors for Il-12 or interferon-gamma

6
Q

Dect in Th 17 diff.

A

Function Def:Decreased T cell-mediated macrophage activation, susceptibility to infection

Mech of Def. Rare, due to mutation in genes encoding, STAT3, IL-17, IL-17R

7
Q

X-linked lymphoproliferative syndrome

A

Function Def:Uncontrolled EVB- induced B cell proliferation and CTL activation, defective NK cell and CTL function antibody responses

Mech of Def. Mutations in gene encoding SAP ( adaptor protein involved in lymphocyte signaling)

8
Q

Human immunodeficiency virus infection

A

Mech: depletion of CD4 helper cells

9
Q

Irradiation and chemotherapy treatments for cancer

A

Mech:Decreased bone marrow precursors for leukocytes

10
Q

Immunosuppression for graft rejection and inflammatory diseases

A

Mech: Depletion or functional impairment of lymphocytes

11
Q

Involvement of bone marrow by cancer( metastases, leukemias)

A

Mech: Reduced site of leukocyte development

12
Q

Protein-calorie malnutrition

A

Mech:Metabolic derangements inhibit lymphocyte maturation and function

13
Q

Removal of spleen

A

Mech: Decreased phagocytosis of microbes

14
Q

X-linked SCID

A

Func Def:Markedly decreased T cells or increased B cells, reduce serum Ig

Mech: Cytokine receptor common gamma chain gene mutations, defective T cell maturation due to lack of IL-7 signals

15
Q

Autosomal Recessive SCID

A

Func Def:Progressive decrease in T and B cells (mostly T)

Mech: ADA or PNP def lead to accumulation of toxic metabolites.

Or can be due to defect maturation of T and B cells due to mutation in RAG genes messing up VDJ recombination

16
Q

X linked agammaoliema

A

Func Def: Decrease in all serum Ig isotypes: reduced B cell numbers

Mech: Block in maturation beyond pre-b cells, because of mutation of BTK

17
Q

Ig heavy chain deficiencies

A

Func Def: Def. of IgG subclasses, associated with absent IgA or IgE

Mech:Chromosomal deletion involving the heavy chain (14q32)

18
Q

DiGeorge Sydrome

A

Func Def:Decreased T cells: normal B cells, normal or decreased Ig

Mech: Anomalous development of 3rd and 4th branchial pouches leading to thymic hypoplasia

19
Q

Wiskott Aldrich Syndrome

A

x-linked recessive disease

Mutation in the WAS gene( cellular cytoskeleton)

Platelets and leukocytes are small and fail to migrate

20
Q

Ataxia-telangiectasia

A

Gait abnormalities

Mutation in gene involved in DNA repair

Defective lymphocyte maturation

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