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55 Cards in this Set

  • Front
  • Back
If you have an immune disorder that is CELLULAR what is your problem?
**white text
T cells

problems with infections
If you have an immune disorder that is HUMORAL what is your problem?
antibody (B cell)
what are Primary Immunodeficiencies
Congenital/genetic; rare
Typically manifest in childhood
what is Bruton X-linked agammoglobulinemia
Lack of mature B cells

Presents in infancy

Virtually absent serum Ig

Normal cell mediated immunity

Recurrent bacterial infections
Encapsulated bacteria (e.g., Strep. pneumoniae)
Are T cells effected in Bruton X-linked agammoglobulinemia
HELL NO!

damn alligator.

only B cells
describe CVID (common variable immunodeficiency)
Heterogeneous group of diseases; may be acquired; no uniform etiology / etiology unclear

Generally lack all Ig’s

Similar presentation to Bruton’s,BUT LATER IN LIFE!!(childhood or adolescence)
Prone to developing lymphoid malignancies
describe Isolated IgA deficiency
Most common primary immunodeficiency
1/600
May be acquired s/p infection

Almost complete lack of IgA

Often asymptomatic
May have recurrent sinopulmonary infections; allergic airway disease; autoimmune disease

~40% pts have IgA autoantibodies
Important consideration for blood transfusions

Recipient circulating anti-IgA antibodies can react with IgA in donor serum  transfusion reaction
what is the most common primary immunodeficiency?
Isolated IgA deficiency
If blood was cross matched, typed, and everything seemed okay,and you saw a transfusion reaction, what might you start thinking?
~40% pts have IgA autoantibodies
Important consideration for blood transfusions
Recipient circulating anti-IgA antibodies can react with IgA in donor serum  transfusion reaction
describe DiGeorge Syndrome
Variable severity; typically present in infants

Failure of normal development of the 3rd or 4th pharyngeal pouches:
Includes thymus, parathyroids, some cardiac structures) -->hypoplastic thymus

Lack of mature T cells

Increased susceptibility to fungal, viral, protozoal infections (some bacteria as well)
Parathyroid hypoplasia: hypocalcemia --> tetany
pt. presents with hypocalcemia, cardiac anomalies, and recurrent infections

what does this patient have?
DiGeorge Syndrome
describe SCID (severe combined immunodeficiency)
Group of disorders that affect both T cells and B cells
Severe, recurrent fungal, viral, protozoan, and bacterial infections
what is an iatrogenic immunodeficiency?
Immune suppression in transplant patients (kidney; heart)

Cancer treatment
Suppression of bone marrow with leukopenia

this is when medicine suppresses your immune system
Systemic, chronic disease example please


Secondary (Acquired) Immunodeficiency
E.g., malignant neoplasm
HIV – AIDS

what is depleted?
what kind of infection is this?
increased risk of what?
CD4+ T cell depletion

Opportunistic infections: agents not typically infectious in immune-competent persons

increased risk of getting malignant neoplasms (B cell lymphomas; Kaposi's sarcoma)
Type I HS Rxn

Ab?
: immediate-type

Antibodies targeting Ag’s already are circulating when the Ag exposure occurs

IgE antibody “coating” mast cells and/or basophils binds to Ag and results in degranulation (Ag cross-links Ab “receptors”)

Localized: urticaria, asthma
Generalized / severe: anaphylaxsis
Type II HS Rxn

Ab?

plz list the 2 types of effects
IgG and IgM

Cytotoxic effects: Ab binds to cell surface antigens and irreversibly damages cells via complement-mediated pathways
E.g., autoimmune hemolytic anemia; Goodpasture disease

Noncytotoxic: Ab’s against cell surface receptors; no cellular destruction
E.g., Graves hyperthyroidism, myasthenia gravis
Type III HS Rxn

ab?
immune complex disease

IgG, IgM, IgA

Ab’s complex with either circulating Ag or with Ag that is deposited in tissues
Immune complexes elicit inflammation via complement activation
Directed against exogenous or endogenous Ag
Autoimmune diseases; serum sickness
Collage-vascular diseases
SLE; RA (rheumatoid arthritis); types of glomerulonephritis; types of vasculitis
Type IV HS Rxn
cell-mediated; delayed type

Greatest intensity 24-48 hrs after exposure

Involves immune response to complex Ag’s; Ag-presenting cells

T cell-mediated tissue injury (CD4+ and CD8+)

Some forms of contact dermatitis (e.g., poison ivy); some autoimmune inflammatory processes (e.g., type I DM; chronic thyroiditis; primary biliary cirrhosis)

PPD rxn. In TB skin test

Impaired T cell-mediated immune rxns. (e.g., as in AIDS) attenuates type IV rxns (anergy)
exudate
high protein high cells

accute inflammatory

Neutraphils present
review virchow's triad
stasis: interrupted blood-flow, change to turbulent blood flow

endothelial injury

hypercoagulability
people with LUPUS have what lab finding
elevated PT and PTT

even though they have blood clots
say a person has none of the standard signs of blood clotting,
genetic mutation in factor V leidien gene mutation
Nickel contact dermatitis is what type of HS rxn?
Type IV
Graves disease is an example of what HS rxn (be specific)
non-cytotoxic type II HS rxn

Ab acts like TSH to hyperstimulate the thyroid
The PPD rxn in the TB skin test will show what type of HS rxn?
Type IV
What is Anergy *** ON THE TEST!!!
occurs when an individual lacks the appropriate T-cell component for delayed cell hypersensitivity rxn

Pt will get negative PPD test, so it will look like they don't have TB

This is what happens in AIDS
anaphylaxis is what kind of HS?
Type I
Autoimmune hemolytic disease

HS type?
type II
Goodpasture's disease is what type of HS rxn?
type II
Lupus is what type of HS rxn?
Type III
Multiple sclerosis is what type of HS rxn?
IV
if sperm leave the privileged area in the testis, what can they act as?
antigen, they will be destroyed
if you have an imbalance of CD4/CD8 imbalance of activity what would you expect to see?
autoimmune disease
who are affected more readily in autoimmune diseases, Males or Females?
Females

sorrrrryyyy
what is lupus in general?
Chronic autoimmune disease affecting multiple organs / systems
if you see someone with renal problems and a slew of others such as Skin, joint,respiratory, cardiac, CNS, serous membranes

what are you thinking?

this could be on the test
LUPUS
if you see antibodies targeted against double-stranded DNA what would you be thinking?
SLE

LUPUS
if you see a malar rash, what are you thinking
oh shit its that old g

LUPUS!
what is Libman-Sacks Endocarditis ?

what is it seen in?

what is the buzzword you will see with this?
endocarditis of the mitral valve in lupus erythematosus

Buzzword: Vegetation

vegetative responses
Sjogren Syndrome
Chronic autoimmune disease resulting from destruction of lacrimal and salivary glands
Patient comes in complaining of dry mouth and dry eyes

you see keratin in the conjuctiva

what does this person have?
Sjogren Syndrome
Scerloderma
Systemic sclerosis

Excessive systemic fibrosis: skin, GI tract, kidneys, heart, muscles, lung
you see a patient with fibrosis in the skin and cyanosis of the tips of fingers associated with Raynaud phenomena. What might you investigate further as a possible disease?
Scerloderma
if you have more localized scleroderma, what would you have?
CREST syndrome

Calcinosis;
Raynaud phenomenon; esophageal dysmotility; sclerodactyly;
telangiectasia
what is CREST syndrome?
Calcinosis;
Raynaud phenomenon;
esophageal dysmotility;
sclerodactyly;
telangiectasia (small dilated blood vessels near the surface of the skin or mucous membranes)

localized sclerosis
what type of HS rxn are seen in transplant rejection
both Type II (humoral rejection)

and type IV (cell-mediated delayed)
what is hyperacute rejection?
tissue rejection that occurs within minutes or hours

Rapid cyanosis, congestion, mottling – immediate graft failure
Ag-Ab rxn at endothelial cell (type II HS rxn)
Deposition of Ig, complement: fibrin-rich thrombi
Action of preformed host Ab’s against donor Ag (ABO Red cell or HLA antigens)
a patient is in surgery getting a new kidney. The doctor removes the clamps to allow blood from the host to mix with the new kidney. The surgeon then sees rapid cyanosis, congestion, and mottling.

The surgeon says damn he is having...
hyperacute rejection
what is acute rejection of tissue?
most common type of rejection to occur

happens in 7-10 days or months/yrs depending on use of immunosuppression

Humoral (type II HS rxn) or cellular (type IV HS rxn), or both
if you have cellular acute rejection, what is the immune system targeting?
endothelial cells

will see Endotheliitis
Generally responds well to immunosuppressant tx (e.g., cyclosporine)
Precursor to chronic rejection
the humoral response of acute rejection does what?
Ab target donor blood vessels
Necrotizing vasculitis
Responds poorly to tx
Chronic cellular rejection

time frame?
symptoms?
therapy?
As early as 2 months post txp, but usually >1 yr

Severe organ dysfunction, with fibrosis
Arteriopathy

Unresponsive to therapy; graft will fail
what is Graft vs. Host Disease (GVHD)

what kind of transplant is this seen in ?
Donor immune cells target host cells

seen in bone marrow transplant
a patient has recently had a bone marrow transplant and is now experiencing Rash, diarrhea, cramping/abd pain, liver dysfunction

what is going on?
Graft vs. Host Disease (GVHD)