Immune Deficiencies Flashcards

1
Q

Infections in Pure B cell deficiency

A

antibody is not made

chronic bacterial infections (pneumonia and diarrhea)
from “high grade”, extracellular, bacterial pathogens- Staph aureus, Haemophilus influenza and Strep pneumonia.

Susceptible to enteroviruses (polio) entering mucous membranes because no IgA

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2
Q

Infections in Pure T cell deficiency

A

severe intracellular infections of viruses, certain bacteria and yeasts and fungi.

Common infections are Candida albicans and Pneumoncystis carinii.

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3
Q

DiGeorge Syndrome

A

Deletion of Chromosome 22–> 3rd and 4th pharyngeal pouches (arches) to develop abnormally

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4
Q

Symptoms of Digeorge

A

immune deficiency from abnormal or absent stromic cells in the Thymus

no parathyroid glands –> hypocalcemia

large blood vessels malformed

abnormal facies (low ears, fish mouth, cleft palate)

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5
Q

incidence of selective IgA deficiency

A

Most common immune disease

1/400-500

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6
Q

Selective IgA Deficiency syndromes

A

mostly asymptomatic because IgM takes over IgA

if IgM insufficient

1) severe allergies
2) infection of mucosa (diarrhea, sinopulmonary infection)

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7
Q

Immunological problem of Nude mouse

A

Fail to make thymic stroma

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8
Q

name the human immunodeficiency condition nude mice resembles

A

no T cells

DiGeorge Syndrome

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9
Q

Name the enzyme which is absent in some cases of SCID

A

autosomal recessive cases of SCID –> absent functioning of the enzyme adenosine deaminase (ADA).

Adenosine accumulates in all cells
Toxic to lymphoid cells so can’t develop
Absence of lymphoid derived cells.

mutation to the Rag V(D)J recombination machinery
inability to arrange the heavy and light chains of B cells and the alpha and beta chains of T cells

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10
Q

Treatments for SCID

A

Purified ADA

Bone marrow transplantation has about a 50% success rate, but graft vs host disease is an ongoing problem

purified stem cells

gene replacement therapy but can cause leukemia

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11
Q

transplantation therapy in immunodeficiency diseases

A

Thymic transplantation for DiGeorge patients by harvesting fetal thymus or culture thymic stromal cells
Minimize graft vs host with cultured thymic stromal cells

Bone marrow transplantation for SCID (50% success)
Minimize graft vs host with purified stem cell

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12
Q

Test for B cell deficiency

A

quantitating antibody levels (IgG, IgA and IgM)
(measuring “titers” or levels of a specific antibody to prior immunizations )

testing ABO isohemagglutinins

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13
Q

Test for T cell deficiency

A

total lymphocyte count

skin tests with antigen panels

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14
Q

Complement tests

A

the CH50 test which tests the integrity of the entire classical pathway

assay for C1 inhibitor

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15
Q

Phagocyte tests

A

WBC count with differential

peripheral smear morphology

NBT test to test for functional oxidative bursts

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16
Q

T and B cell areas in lymph node

A

Outer Cortex made of Follicles = B cells

Germinal Center = Dividing B cells

Medulla = B cells


Paracortex = T cells

17
Q

Describe the contents and routes of administration of commercial gamma globulin

A

Human gammaglobulin given monthly via IV,
New subq infusion at home
Immunoglobulin is clean (few broken molecules) and composed almost entirely of IgG with a little IgA

18
Q

Conditions to use IVIG

A

Any immune deficiency where there is deficient B cell function (SCID, Brutons, CVID

19
Q

Problem with IVIG

A

contain small amounts of IgA

bad for selective IgA deficiency because IgA can be recognized as foreign –> immune reaction

20
Q

two viruses which are immunosuppressive in humans

A

measles, Mononucleosis and CMV

In mono, herpes viruses (such as EBV) infect B cells, causing T cells to destroy the infected B cells

HIV destroys helper T cells by forming syncytia.
Patients can’t fight off intracellular viruses, bacteria and fungi