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2. Immunology > Immunodeficiency Diseases > Flashcards

Immunodeficiency Diseases Flashcards

1
Q

Immunodeficiency diseases

A

Loss in function of parts of the immune system that causes the immunodeficient patient more susceptible to infectious diseases

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2
Q

What are the 2 types of immunodeficient states?

A

Primary and secondary

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3
Q

Primary immunodeficient state

A

Mutation in a gene encoding molecule of the immune system: an inherited or congenital disease
Inhibits development of both B and T cells

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4
Q

Secondary immunodeficient state in an adult animal

A

Due to age, malnutrition, infection, neoplastic or other chronic inflammatory diseases or due to the administration of immunosuppressive drugs

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5
Q

Recognition of immunodeficiency diseases in patients

A
  1. Disease occurring in young littermate animals with onset shortly after the expected time of loss of maternally derived immunity
  2. Disease affecting a particular breed
  3. Chronic and recurring infections
  4. Unusual infectious agents
  5. Incomplete response to treatment
  6. Failure of infection to respond to standard antimicrobial therapy
  7. Infection with environmental saprophytes
  8. Persistant lymphopenia or hypogammaglobulinemia
  9. Failure to respond to vax
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6
Q

Sever Combined Immune Deficiency Disease (SCID)

A

Fatal disease where poor B and T cell development results in lymphopenia, marked serum IgM and IgA deficiency, and hypoplasia of lymphoid tissues (2/3 to diagnose)

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7
Q

What causes SCID?

A

Lack of activity of the enzyme DNA-PK from a deletion mutation of the gene encoding the catalytic subunit

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8
Q

DNA-PK

A

Required for gene rearrangement of the antigen-receptor on B and T cells

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9
Q

What is the mode of inheritance of SCID?

A

Autosomal recessive

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10
Q

How do you diagnose SCID?

A

DNA test of whole blood or cheek swab
Should be performed in all Arabian and Arabian-crossbred horses

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11
Q

Phenotype of SCID

A

SCID foals are normal @ birth but show symptoms of increased temp, resp stress, and diarrhea (2-8 weeks of age)

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12
Q

Genotype of SCID

A

N/SCID = carriers (50% of offspring) and mating between 2 carries is 25% of affecting foal
SCID/SCID= fatal

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13
Q

Treatment and prognosis of SCID?

A

No treatment, die @ 4-6 months
Abscessation of the lung
Lymphoid tissue hypoplastic

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14
Q

Where is canine SCID recognized?

A

Basset hound, Cardigan Welsh Corgi, Jack Russel Terrier
XSCID fatal in humans by 2 and in dogs by 4 months unless treated with bone marrow transplantation

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15
Q

IgA deficiency

A

Most common human primary immunodeficiency disorder
Normal lives with chronic recurrent mucosal infections or have autoimmune, allergic or neoplastic diseases
Deficiency in IgA production

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16
Q

Equine Ig infections

A

Selective IgM deficiency, IgG deficiency and primary agammaglobulinemia (absence of B cells and all Igs)

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17
Q

What happens to foals with selective IgM or IgG deficiencies?

A

Develop recurrent infections of mucosal surfaces

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18
Q

What happens to adult horses (<3) with selective IgM or IgG deficiencies?

A

Recurrent infections, low to undetectable concentration of serum Igs and undetectable B cells

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19
Q

Fell Pony Syndrome

A

Inherited in autosomal recessive
Immunodeficiency disorder with deficiency of B cells and plasma cells
Mutation of chromo ECA26

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20
Q

Clinical signs of Fell Pony Syndrome

A

Anemia and respiratory enteric infections
Loss of maternally derived Ab and affected foals die by 3 months

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21
Q

Thymic Aplasia

A

Consequent lack of T cells
Linked to failure of development of hair coat

22
Q

Kittens with thymic aplasia

A

Born with an autosomal recessive form of congenital hypotrichosis (thymic aplasia-nude kittens)

23
Q

Zinc-associated disorders

A

Lymphocyte dysfunction and subnormal plasma zinc levels (reduced T cell mitogen responsiveness and low serum IgA concentration)

24
Q

Lethal acrodermatitis

A

Zinc-associated disorder
Bull terrier dogs with autosomal recessive disease
Dogs have stunted growth, severe cutaneous parakeratosis and skin and resp infections

25
Lethal train A46 or Bovine hereditary zinc deficiency
Autosomal recessive disorder in black pied Danish and Friesian cattle Also a defect in T cell immunity related to reduced ability to absorb dietary zinc
26
Signs of Lethal train A46
Skin lesions @ 4-8 weeks of age without treatment Will die within 4-8 weeks of clinical onset
27
What causes Lethal train A46?
A mutation in the SLC39A4 gene (encodes a zinc intestinal transporter) prevents normal absorption of zinc from the intestinal tract
28
Gray Collie Syndrome (cyclic hemtopoiesis)
Cyclic fluctuations in leukocyte numbers in collie pups, poorly grown, diluted grey coat color Autosomal recessive Mutation of gene encoding subunit of the neutrophil adaptor protein complex 3
29
What happens to neutrophils during gray collie syndrome?
Neutrophil count normal for 11-12 days, low for 3 Neutropenia, monocytopenia, thrombocytopenia and reticulocytopenia
30
How is Gray Collie Syndrome treated?
By administration of granulocyte colony stimulating factor o stem cell factor by bone marrow transplant
31
Pelger Huet Anomaly (neutrophil disorders)
Failure of nuclei of neutrophils to segment into lobes Reduced chemotaxis die to inflexible nuclei
32
What breeds is Pelger Huet Anomaly (neutrophil disorders) found in?
Cocker spaniels, basenjis, boston terriers, fox-hounds, coon-hounds, australian shepherd dog, domestic short hair cat
33
Chediak Higashi syndrome
Abnormal granulation of the neutrophils cytoplasm Autosomal recessive with defective neutrophil function and increases susceptibility to infection
34
What is Chediak Higashi syndrome caused by?
By a mutation in the LYST gene, which encodes a molecule involved in the fusion of lysosomal membranes
35
What species is affected by Chediak Higashi syndrome?
Blue smoke Persian cat, Hereford, Japanese black and brangus cattle, Aleutian mink, white tigers, killer whales and man and Birman cats (no symptoms)
36
What signs are shown in animals with Chediak Higashi syndrome?
Diluted coat color and poorly pigmented irises due to fusion of melanosomes Hemorrhages following trauma
37
Trapped Neutrophil Syndrome
Autoimmune recessive in Border collie dogs Most puppies get sick and die by 4-5 months Bone marrow produces neutrophils normally but not transferred in blood, then they're trapped
38
What causes Trapped Neutrophil Syndrome?
TNA mutation of the VPS13B gene
39
Signs of Trapped Neutrophil Syndrome
Abnormally long heads and legs Smaller than other puppies Inability to nurse properly Weakness and depression Frequent illness and infections Swollen joints and leg pain
40
Leucocyte Adhesion Deficiency (LAD)
Autosomal recessive disease In dogs (red irish setter= CLAD) In cattle (holstein and friesian= BLAD)
41
Cause of LAD
Mutation in the gene encoding the a2 leucocyte integrin CD18 Failure to express CD18--> failure to express CD11b --> circulating blood neutrophils can't adhere to vascular endothelium and migrate to tissues
42
What do animals with LAD show?
Fever and anorexia Leukocytosis (neutrophilia) Severe recurrent infections
43
Etiological factors of secondary immunodeficiency
Malnutrition Hormonal imbalances Autoimmune and inflammatory diseases Treatment with immunosuppressive drugs (corticosteroids or cyclosporine) Viral and other infections induce secondary immunodeficiency
44
What diseases in dogs cause secondary immunodeficiency?
Canine distemper, parvo and demodicosis
45
What diseases in cats cause secondary immunodeficiency?
Feline infectious peritonitis, feline leukemia virus (FeLV) and feline immunodeficiency virus (FIV)
46
Feline AIDS (FIV)
Caused by FIV Infects CD4+, macrophages, denderitc cells, astrocytes and CD8+ t cells
47
Immunological defects in cats with FIV
Progressive decrease of CD4+ Decreased function of lymphocytes and macrophages Decreased IL-2 production
48
How id FIV spread?
Through cats saliva Possible but less common to pass FIV to kittens before they're born or while they're nursing
49
How can FIV be prevented?
Soap and water Spaying and neutering cats (minimizing biting behavior and ends transmission from mother to kittens)
50
What are the 3 stages of FIV?
1. Initial infection/ acute phase 2. Asymptomatic phase 3. Potential clinical phase
51
Signs of FIV
Loss of appetite and weight loss Poor coat condition Persistent fever Inflammation of gums/ mouth Seizures, neurological disorders Eye conditions
52
Diagnosis of FIV
Blood samples examined for Abs cats get to fight against the disease ELISA, positive results confirmed using western blot or IFA and PCR

2. Immunology (24 decks)

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