Deficiency of Adaptive Immunity Flashcards

1
Q

Primary Immunodeficiency

A

Congenital

Caused by innate issues with WBC production

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2
Q

Secondary Immunodeficiency

A

Acquired

When infection or radiation paralyzes immune system development

Measles, malaria, EBV (infectious mononucleosis)

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3
Q

Infections of Pure B cell Deficiency

A

High grade bacterial pthogens like Staph A, Haemophilus I, and Strep P.

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4
Q

Severe Combined Immunodeficiency (SCID)

A

Rare, both T and B immunodeficiency. Of the lymphoid stem cell.

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5
Q

DiGeorge Syndrome

A

Failure of 3/4 pharyngeal pouches to develop into a thymal stroma

No T cell maturation

Also presents with: cardiac defects (tetralogy of flow), parathyroid deficieny (convulsions due to hypocalecemia)

Chromosomal deletion on Chr 22

Fungi and viruses

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6
Q

X-linked Bruton’s hypogammaglobinemia

A

Failure to b cell differentiation

Pre-B cell arrest, with IgM in cytoplasm

Defect: BTK (Bruton’s Tyrosine Kinase) used for B cell maturation

Can’t make antibody, so that’s the treatment

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7
Q

SCID-X1

A

X linked, Most common
no production of protein Gamma C, part of IL-2 receptor, shared by 7 known cytokine receptors, including IL-7
IL-7 Critical for development of T and B cell

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8
Q

SCID- ADA Deficiency (Adenosine Deaminase)

A

Adenosine accumulates in all cells, but it is necessary for developing lymphocytes, so they die

Treatment: give them the missing enzyme, or just blood transfusions, because RBCs are full of Adenosine Deaminase (irradiated to kill T Cells)

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9
Q

V(D)J Recombination Deficiencies

A

SCID A- highest incidence in athobaskan indians.
Artemis defect- enzyme involved in looping and cutting looping from VDJ recombination

RAG Enzyme- Some mice are rag knockouts

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10
Q

Nude Mice

A

Fail to make thymic stroma, so no T cells, so they look like DiGeorge Kids

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11
Q

X-linked Hyper IgM syndrome

A

When T follicular helper or B cell is mutated, and IgM can’t switch to IgG

CD40 mutation on either cell

Immunodeficiency because limitations of IgM

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12
Q

Common Variable Immunodeficiency (CVID)

A

Can have this and patient can be mostly asymptomatic, and then sudd
enly can’t respond to new challenges

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13
Q

DiGeorge Clinical Manifestations (non immunological)

A

Fish (arched) mouth, wide spread eyes, low set ears,

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14
Q

T Cell Deficiency Associated Infections

A

Intracellular pathogens like viruses, certain bacteria, yeast and fungi, especially Candida albicans and Pneumocystis jirovecii

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15
Q

Selective IgA Deficiency

A

Seems to be able to secrete IgM into mucosa instead, otherwise mostly assymptomatic

Diarrhea and sinoppulmonary infections, or increased allergies

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16
Q

Transplantation Treatment

A

You can transplant fetal thymi or thymus stromal cells (for DiGeorge), or Bone marrow transplants for SCID

Complications- Graft v host disease

In the thymus, that’s where t cells learn MHC recognition, so matching is crazy imperative

17
Q

IVIG Treatment

A

Great for B cell deficiency

Given monthly, 99% IgG, you have to do it in an infusion center, for risk of immunological reaction (sudden inflammatory problem)

Subcutaneous is optional and doesn’t have to be done at a transfusion center

18
Q

Name two viruses which are immunosuppressive in humans. Discuss a possible mechanism for the immunosuppression caused by one of these viruses.

A

HIV and Measles, malaria, EBV (infectious mononucleosis)

HIV targets CD4.

19
Q

B Cell Deficiency Test

A

Serum electrophoeresis, Ig levels, specific Antibodies

Advanced: antibody response to new antigen, gene sequencing

20
Q

T Cell Deficiency Tests

A

Skin tests with recall antigen, or total lymphocyte count

Advanced: CD3,4,8 count, mitogen response

21
Q

Phagocyte Deficiency Test

A

WBC Count, diff, smear, NBT test

Advanced: Assay for phagocytosis/chemotaxis, genetics

22
Q

Complement Deficiency Test

A

Chem50 test, C1 inhibitor assay

Advanced: Individual complement component evels