deck_21100043 Flashcards by Jorge Torres

What is a primary immunodeficiency?

An immunodeficiency resulting from an inherited genetic defect in the immune system

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What is a secondary immunodeficiency?

Loss of immune function that results from exposure to an external agent

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Primary immunodeficiencies are more dangerous and life-threatening than secondary immunodeficiencies. True or false?

False. AIDS is a secondary immunodeficiency. They are both as dangerous or as benign

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The most common type of immunodeficiencies are antibody disorders. True or false?

True

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Combined immunodeficiencies are what happens when someone with PID gets a SID. True or false.

False. Absence of T cells or significantly impaired T cell function, combined with the disruption of Ab responses

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Severe combined immunodeficiency targets steps that occur in early B/T cell development. True or false?

True, but it can target early hematopoiesis

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Despite being called “combined”, SCID are the result of an extreme secondary immunodeficiency. True or false?

False. it’s the result of a gene defect, usually in the gamma chain.

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Some genetic defects that can lead to SCID are ADA deficiency or AK2 deficiency. What do these deficits cause?

ADA results in the accumulation of toxic purine degradation byproducts, which affects mostly lymphocytes.

AK2 leads to the loss of the AK2 protein in mitochondria, which is responsible for maintaining the homeostasis of AMP, ADP and ATP by catalyzing the transfer of P. This results in a block on both myeloid and lymphoid lineages.

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Mention 6 gamma chain cytokines.

IL-2, IL-4, IL-7, IL-9, IL-15, IL-21

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BLS I is caused by the absence of MHC I and leads to enhanced susceptibility to viral infections. True or false?

True

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BLS II is caused by mutations and causes problems in MHC II. True or false?

True

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What does WASP do?

Nests. Jk

It regulates the formation of actin filaments in hematopoietic cells.

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A patient was found with normal T cell numbers but impaired functions. When he was found a couple of years later, he was much worse. What did he have?

Wiskott-Aldrich syndrome.

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A patient presented typical WAS symptoms:
- She had normal T cell numbers, but impaired functions
- reduced IgM concentrations
- Normal IgG levels
- Elevated IgA and IgE levels

Something is wrong in this description. What is it?

The pronoun. WAS only affects males.

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A patient presented typical WAS symptoms:
- He had normal T cell numbers, but impaired functions
- reduced IgA and IgE concentrations
- Normal IgG levels
- Elevated IgM levels

Something is wrong in this description. What is it?

The IgA and IgE levels are switched with IgM

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A patient presented typical WAS symptoms:
- He had normal T cell numbers, but impaired functions
- reduced IgM levels
- Normal IgD levels
- Elevated IgA and IgE levels

Something is wrong in this description. What is it?

Nothing is wrong per say, but it should read “normal IgG levels”

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What are the mechanisms of disease in WAS?

Decreased T cell effector function because of problems in antigen presentation by APCs
Defective B cell responses

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DiGeorge syndrome is related to problems with developing the thymus. True or false?

True

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Even in the most extreme cases of DiGeorge syndrome, a little bit of thymus is developed. True or false?

False. Complete DGS results in a severe reduction of T cell numbers and poor Ab responses.

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DIGEORGE SYNDROME

Thymic transplantation and passive antibody treatment are enough to correct the problems caused by this syndrome. True or false?

False. This syndrome also causes congenital heart abnormalities.

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FOXP3 deficiency, despite causing problems in Thymus development, doesn’t actually cause immunodeficiency problems. True or false?

False. Of course it does.

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How is hyper-IgM syndrome created?

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It’s a defect in CD40-L or CD40

How does Hyper-IgM syndrome increase the production of IgM?

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No CD40 —> No heavy chain class switching —> No switch means “the default” is the only one that can be produced —> Only IgM is produced

B cell immunodeficiencies are associated with susceptibility to viral infection. True or false?

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False. Its bacterial infections.

What causes XLA and what are the consequences?

A lack in Btk, which is necessary for transducing signals from a pre-BCR and BCR. As a result, patients have no peripheral B cells.

If I examine a CVID patient's T cell levels, what can I expect to see?

Nothing out of the ordinary.

If I examine a CVID patient's B cell levels, what can I expect to see?

Nothing out of the ordinary. BUT I will notice that there are no plasma cells and no antibodies, as CVID B cells are unable to differentiate into plasma cells.

Defects in the myeloid lineage affect the adaptive immune system. True or false?

False. It affect the innate immune system.

Chronic Granulomatous Disease is the result of an inability to ________________________________________________________ because of defects involving _______ _________________.

generate activated forms of oxygen NADPH oxidase system

Chronic Granulomatous Disease is directly related to ATP. True or false?

False.

What cells are most affected by CGD?

Any cell that needs to produce reactive oxygen species, which includes monocytes, macrophages and neutrophils.

What happens in Chediak-Higashi syndrome?

Accumulation of giant cytoplasmatic granules due to the incapacity of lysosomes fusing with phagosomes, which leads to impaired phagocytized bacteria.

What cells are affected by Chediak-Higashi syndrome?

Neutrophils, NK cells and Cytotoxic T lymphocytes.

A deficiency in the complement lineage all lead to the same outcome, which is?

C3 convertase is not formed, C3 is not cut, and the complement-mediated response is fucked.

What are three treatments for immunodeficiencies?

Replacement of missing protein Replacement of missing cell type Replacement of missing or defective gene | If you said "complete isolation" istg I'm killing you

What advantages does gene therapy have over HSC grafts?

No donor needed No GVHD One time correction instead of a lifelong therapy.

What is the function of the ENV spike/gp120?

Bind to the target receptor (CD4) or coreceptor (CXCR4 or CCR5)

What are the three enzymes present inside HIV?

Reverse transcriptase Protease Integrase

HIV is extremely efficient as all it needs is to bind to CD4 for the virus to infect a cell. True or false?

False. After gp120 binds to CD4, it needs to bind to a coreceptor for the virus to infect the cell.

What are the differences between T-Tropic and M-Tropic HIV?

The cells that they infect and the coreceptors they bind to. T-tropic binds to central memory and naive T cells and uses the coreceptor CXCR4 M-Tropic binds to macrophages, DCs, and effector memory T cells and uses the coreceptor CCR5

Only X4 (T-Tropic) HIV binds to T cells. True or false?

False. M-tropic (R5) binds to effector memory T cells

How can HIV infection be blocked?

Since HIV uses coreceptors to infiltrate the cell, the natural ligands of said coreceptors compete with HIV for the coreceptors, which can end up blocking the infection.

What is the HIV variant that spreads between people?

It's the R5 variant, aka the one that uses the CCR5 coreceptor.

The CCR5 Δ32 mutation confers resistance to HIV by changing the binding site of the receptors, thus making it so HIV can't bind to it. True or false?

False. It actually leads to less expression of CCR5 (since the missing genes lead to a non-functional protein) and, with no CCR5, HIV can't bind to it.

After about 12 weeks of initial HIV infection, there seems to be an improvement. Why is that?

Because the blood-borne virus is reduced to very low levels and remains this way for many years, but it's too late. The patient is already infected, which leads to a steadily decline of CD4+ T cells. They decline because of active viral replication and T cell infection in lymph nodes.

Progression of HIV infection

1. Acute infection partially controlled by adaptive immune response 2. Infection of memory CD4 T cells in the mucosal lymphoid tissues and death of many cells 3. Dissemination of the virus, viremia, and the development of host immune response 4. Chronic infection. Lymph nodes and the spleen are sites of continuous HIV replication and cell destruction

If we could somehow find a way to control the acute infection, we could stop HIV from infecting cells. True or false?

False. The immune response of the host temporarily controls the acute infection but is unable to prevent the establishment of chronic infection in cells in the lymphoid tissues.

Its only considered as "Having AIDS" when a person is in the latest phase of the disease, this being the depletion of CD4 T cells and the destruction of lymphoid tissue. True or false?

True

Most T cells undergoing apoptosis in HIV infection are uninfected. True or false?

True

HIV progression (and CD4 cell loss) can be quickened by infections of other microbes in HIV patients. True or false?

True

Name 3 proposed contributors to HIV associated chronic immune activation, and name which is the most important of them all.

CHRONIC IMMUNE ACTIVATION Altered balance of T cell subsets Microbial translocation Co-infections Persistent elevation of IFN-I and IFN-II HIV-1 replication

What would be needed for an effective HIV vaccine?

Broadly neutralizing antibodies and robust T-cell responses.

Why is HIV so difficult to cure?

HIV can remain "hidden" in latent reservoirs, this is, hidden inside cells where the virus is integrated but transcriptionally silent The cells they prefer to "hide" in are long-lived memory CD4 T cells, which have a long lifespan and capacity for clonal expansion)

deck_21100043 Flashcards

(53 cards)