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Flashcards in Deficiency of Adaptive Immunity Deck (22)
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Primary Immunodeficiency


Caused by innate issues with WBC production


Secondary Immunodeficiency


When infection or radiation paralyzes immune system development

Measles, malaria, EBV (infectious mononucleosis)


Infections of Pure B cell Deficiency

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


Severe Combined Immunodeficiency (SCID)

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


DiGeorge Syndrome

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


X-linked Bruton's hypogammaglobinemia

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



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


SCID- ADA Deficiency (Adenosine Deaminase)

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)


V(D)J Recombination Deficiencies

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


Nude Mice

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


X-linked Hyper IgM syndrome

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


Common Variable Immunodeficiency (CVID)

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


DiGeorge Clinical Manifestations (non immunological)

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


T Cell Deficiency Associated Infections

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


Selective IgA Deficiency

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

Diarrhea and sinoppulmonary infections, or increased allergies


Transplantation Treatment

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


IVIG Treatment

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


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

HIV and Measles, malaria, EBV (infectious mononucleosis)

HIV targets CD4.


B Cell Deficiency Test

Serum electrophoeresis, Ig levels, specific Antibodies

Advanced: antibody response to new antigen, gene sequencing


T Cell Deficiency Tests

Skin tests with recall antigen, or total lymphocyte count

Advanced: CD3,4,8 count, mitogen response


Phagocyte Deficiency Test

WBC Count, diff, smear, NBT test

Advanced: Assay for phagocytosis/chemotaxis, genetics


Complement Deficiency Test

Chem50 test, C1 inhibitor assay

Advanced: Individual complement component evels