Lecture 16: Immuno Deficiency Syndromes

Question 1

Deficiency in IgA leads to deficiency in dealing with pathogens at _____ surfaces.

Answer 1

MUCOSAL

Question 2

Primary (inbron) immunodeficiencies can be genetic or _____ disorders. For example ____-linked SCID.

Answer 2

DEVELOPMENTAL

X-linked SCID

Question 3

Secondary (acquired) immunodeficiencies is the result of a disease process that is not inborn. The most prevalent cause world-wide is ______-______ malnutrition. Also, individuals that undergo radiation or _____-therapy for cancer are immunosuppressed. If cancer metastasizes to _____, this can also lead to a secondary deficiency, because cancer cells crowd out and provide signals that turn off normal _______.

Answer 3

PROTEIN-CALORIE malnutrition.

CHEMOtherapy

BONE

HEMATOPOEISIS

Question 4

Patients with immunodeficiency are typically recognized on the basis of increased _______ to infections, infections with organisms of low ________, or infections of unusual ______.

Answer 4

SUSCEPIBILITY

VIRULENCE

SEVERITY

Question 5

Infancy is a time of ______ immundeficiency in that the child is not receiving protection from maternal Igs, and has not yet produced much of its own. Ig___ is the first one the infant produces, while Ig___ and Ig___ levels rise later as the child ages.

Answer 5

TRANSIENT

IgM

IgG and IgA

Question 6

Aging also results in immunodeficiency for a few reasons. Some examples are, _____ shortening in T-cells , leading to cellular senescence, poor _____ quality, or skewing toward ____ cells for patients infected with CMV.

Answer 6

TELOMERE

Ab

CD8+

Question 7

Protein-calorie malnutrition can lead to reduced production of IL-__ (required for T-cell proliferation) and IFN-_____ (which helps macrophages to kill pathogen.)

Answer 7

IL-2

IFN-GAMMA

Question 8

Splenectomy is immunosuppressive. The major clinical manifestation is an increased susceptibility to disseminated infection with ______ bacteria (i.e. pneumococcus.) This is likely due to reduced _____ and Ab production of spleen.

Answer 8

ENCAPSULATED

FILTERING

Question 9

There are defects in multiple levels of the immune system resultant from infection with HIV, but the main immune cell that is affected is the CD__ T-cell (the virus uses the CD__ receptor and the CXCR__ or CCR___ co-reeptors to enter into the cell, so monocytes and DCs which express low levels of these receptors can be infected as well.) Initially, it is CD__ cells in the ____ that are lost, followed by those in peripheral ____ and lymph nodes.

Answer 9

CD4

CXCR4

CCR5

CD4 cells in the GUT

BLOOD

Question 10

Some examples of Primary immunodeficiencies are as follows:

1. MHC class I deficiency is characterized by mutations in ___1, ___2, and _____. This results in no CD___ T-cells, and leaves patients susceptible to chronic ____ and skin inflammation.
2. MHC class II deficiency is characterized by a lack of expression of MHC class II molecules. This results in a lack of CD__ T-cells, leaving these patients with a general susceptibility to disease/infection.

Answer 10

1. TAP1, TAP2, and TAPASIN

CD8 T-cells

LUNG

CD4

Question 11

The most severe Humoral Deficiency is X-linked _____________. It is characterized by absence or low levels of ______ globulin in the blood and reduced or absent ___-cells in the peripheral blood and lymphoid tissues. __-cell numbers and maturation are usually normal. This disease is caused by a mutation or deletion of the _____ gene.

Answer 11

AGAMMAGLOBULINEMIA

GAMMA globulin

B-cells

T-cell

Btk (B-cell tyrosine kinase)

Question 12

Hyper-Ig__ syndrome is another example of a humoral deficiency. It is caused by an X-linked defect in the gene encoding _____ expressed on T-cells (which is required for B-cell responses to T-dependent Ag.) This results in a lack of ____ switching and formation of _____ B-cells. Therapy involves Ig replacement.

Answer 12

Hyper-IgM syndrome

CD40L

CLASS switching

MEMORY B-cells

Question 13

SCID is considered a disease of the Cellular immune response. Almost half of the cases are due to deficiency of the common ___-chain of the IL-2 receptor (this is known as X-linked SCID or XSCID) which affects IL-___ signaling (which is critical for B and T-cell development.)

Answer 13

Gamma-chain

IL-7

Question 14

SCID-like deficiencies can arise from a defect in ____-70, which is involved in T-cell signal transduction. Patients present with normal levels of Ig, but cannot undergo ______ switching because CD__ T-cells are nonfunctional.

Answer 14

ZAP-70

CLASS switching

CD4 T-cells

Question 15

DiGeorge Syndrome is a congenital immune disorder that results in defective development of the _____ and parathyroid glands. The absent parathyroid causes abnormal ______ homeostasis and muscle twitching. Peripheral __-cells are absent or reduced in number and do not respond to polyclonal activators. Patients are susceptible to ______bacterial, viral, and fungal infections.

Answer 15

THYMUS

CALCIUM

T-cells

MYCObacterial

Question 16

An example of an immunodeficiency disease that affects innate immunity would be Chronic _______ disease. It is caused by an inability of ______ to kill pathogens they have taken up. This results in intracellular and extracellular infections and _______.

Answer 16

GRANULOMATOUS

PHAGOCYTES

GRANULOMAS

Question 17

The most common genetic cause for Chronic Granulomatous Disease is ___-linked. There is a defect in the phagocyte _______ system, including cytochrome ______.

Answer 17

X-linked

OXIDASE

B558