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-disease in which immune function is partially or totally absent
-primary immunodeficiency
a) immunodeficiency
b) immunosuppression
a) immunodeficiency
-inhibition of the immune system by an extraneous fact
-secondary immunodeficiency
a) immunodeficiency
b) immunosuppression
b) immunosuppression
inherited
a) primary immunodeficiency
b) secondary immunodeficiency
a) primary immunodeficiency
primary immunodeficiencies are classified according to what?
the branch of immune system involved
what are some findings that may point to immuno-deficient state
-unexplained neonatal illness affecting more than one animal in a litter
-illness due to commensal or low pathogenic organism
-recurrent illness
-disease from MLV
what is this disorder?
-autosomal recessive disorder
-dysplastic or immotile cilia due to abnormalities in microtubule organization
-breeds: OES, Dachshunds, Bull Terrier, Springer Spaniel, Doberman
-signs include: poor weight gain, coughing, nasal discharge, bronchopneumonia
ciliary dyskinesia
what is this disorder?
-accompanied by dilution of skin/hair pigment
-cyclic fluctuation in WBC numbers
-disease is due primarily to decreased numbers & function in neutrophils
-recurrent infections
Cyclic hematopoiesis of grey collies
what is this disorder?
-autosomal recessive disease
-incomplete albinism, prolonged bleeding, recurrent infections
-enlarged granules within granulocytes
-species: Persians, foxes, minks, cattle, whales, humans
-diagnosed w/ signs and blood smears
Chediak Highashi Syndrome
Chediak Highashi Syndrome is due to a mutation in ________ gene
CHS1
what disease is this?
-autosomal dominant disorder w/ incomplete penetrance
-described in dogs (foxhounds, Australian shepherds)
-incomplete segmentation of granulocyte nuclei
-impaired ability to extravasate
-dogs are usually asymptomatic
Pelger Huet Anomaly
what disease is this?
-AKA canine leukocyte adhesion deficiency
-autosomal recessive trait described in Irish Setters and Holsteins
-neutrophils have defective respiratory burst
-severe infections: skin, gingiva, etc.
canine granulo-cytopathy syndrome
what disease is this?
-mutation in integral - neutrophils cannot emigrate from blood vessels
-also unable to phagocytose bacteria coated with C3b
-recurrent, severe infections
leukocyte adhesion deficiency
immunodeficiency of Weimaraners
-congenital
-recurrent fevers, diarrhea, pneumonia, pyoderma, osteomyelits
-low levels of IgG, IgA, IgM
-defective neutrophil function
what disease is this?
-Arabian horses
-autosomal recessive
-in 2-3% of all foals
-defect in DNA-dependent protein kinase: cannot "paste" DNA strands after cutting
-no functional V region of B and T cell receptors; thus no functional T or B cells
Severe Combined Immunodeficiency
what is SCIDS?
Severe combined immunodeficiency
-defect in DNA-dependent protein kinase: cannot "paste" DNA strands after cutting
what disease is this?
-x-linked (males only)
-normal B cell level but decrease immunoglobulin
-decreased CD8's
-thymus is small
-mutation in IL-2 receptor
canine SCID
dogs with SCID often die from what vaccine?
distemper MLV
what disease is this?
-no B cells, antibody
-x-linked disorder
-mutation in Bruton tyrosine kinase
-impairs B-cell signaling
-seen in male foals
Agammaglobulinemia
what disease is this?
-breed: Arabian and quarter horses
-low IgM in adults associated with infection, stress, lymphoma
IgM deficiency
what disease is this?
-delayed immunoglobulin synthesis
-lymphocyte numbers are normal
-typically self-limiting
-gotta distinguish this from SCID
transient Hypo-gammaglobulinemia
what disease is this?
-breed: Black pied Danish and Friesan (cattle)
-autosomal recessive
-cannot absorb zinc
-T cell function is impaired
-decreased CMI
-skin abnormalities
Trait A46
Zinc is needed for which hormone?
Thymic hormone
which is inherited?
a) primary immunodeficiency
b) secondary immunodeficiency
a) primary immunodeficiency
what are the 3 classes of primary immunodeficiency?
a) Defects in Mechanical Barriers
b) Defects in Non-Specific Immunologic Defenses
c) Defects in Specific Immunologic Defenses
what should you be thinking when you see the following issues:
-several litter mates affected with unexplained disease
-commensal/low pathogenic organisms causing disease
-recurrent illness
-disease after a MLV
primary immunodeficiency
Ciliary Dyskinesia is seen primarily in:
a) Dachshund, Doberman, Bichon Fries, etc.
b) Grey collies
c) Persians, foxes, cattle, etc.
a) Dachshund, Doberman, Bichon Fries, etc.
Cyclic hematopoiesis is seen in:
a) Dachshund, Doberman, Bichon Fries, etc.
b) Grey collies
c) Persians, foxes, cattle, etc.
b) Grey collies
Chediak Highashi is seen in:
a) Dachshund, Doberman, Bichon Fries, etc.
b) Grey collies
c) Persians, foxes, cattle, etc.
c) Persians, foxes, cattle, etc.
signs: poor weight gain, coughing, nasal discharge, bronchopneumonia
a) Ciliary dyskinesia
b) Cyclic Hematopoiesis
c) Chediak Highashi Syndrome
a) Ciliary dyskinesia
signs: diluted pigments, decreased neutrophil presence, decreased neutrophil function, recurrent infections, changing WBC counts
a) Ciliary dyskinesia
b) Cyclic Hematopoiesis
c) Chediak Highashi Syndrome
b) Cyclic Hematopoiesis
signs: incomplete albinism, recurrent infection, enlarged granules/granulocytes, prolonged bleeding times, photophobia
a) Ciliary dyskinesia
b) Cyclic Hematopoiesis
c) Chediak Highashi Syndrome
c) Chediak Highashi Syndrome
in addition to classical signs, what test can we do to support the diagnosis of Chediak Highashi Syndrome?
blood smear: look for giant lysosomes containing big granules
which of the following is an example of a specific, primary immunodeficiency:
a) Ciliary dyskinesia
b) Cyclic Hematopoiesis
c) Chediak Highashi Syndrome
d) SCID
d) SCID
in which do we see no functional B or T cells and rudimentary lymph nodes without follicles?
a) SCID
b) SLE
a) SCID
which disease is only in males:
a) Equine SCID
b) Canine SCID
b) Canine SCID
what is the difference in mechanism between Equine SCID and Canine SCID?
Equine: DNA-dependent protein kinase defect; cannot reattach strands during recombination; no functional V regions on B and T cells
Canine: defect in the IL-2 receptor
which is importantly decreased in canine SCID?
a) CD4+ TH
b) CD4+ Treg
c) CD8+ CTLs
c) CD8+ CTLs
which is induced?
a) primary immunodeficiency
b) secondary immunodeficiency
b) secondary immunodeficiency
Parvovirus leading to pancytopenia is an example of:
a) primary immunodeficiency
b) secondary immunodeficiency
b) secondary immunodeficiency
what is the most common source of immunodeficiency?
failure of passive immunity transfer
what is the major source of passive immunity?
maternal antibody transfer