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| − | *Primary immunodeficiencies may affect either the [[Innate Immune System - WikiBlood|innate immune system]] or the [[Adaptive Immune System - WikiBlood|adaptive immune system]]
| + | {{frontpage |
| − | *They are categorised by either the type or the developmental stage of the cells involved
| + | |pagetitle =Primary Immunodeficiency |
| − | *Lymphoid cell disorders affect [[Lymphocytes#T cells|T cells]] or [[Lymphocytes#B cells|B cells]] (or both)
| + | |pagebody =Primary immunodeficiencies may affect either the [[Innate Immune System|innate immune system]] or the [[Adaptive Immune System|adaptive immune system]]. They are categorised by either the type or the developmental stage of the cells involved. Lymphoid cell disorders affect [[Lymphocytes#T cells|T cells]] or [[Lymphocytes#B cells|B cells]] (or both). Myeloid cell disorders affect phagocytic function. The severity of the immunodeficiency depends on at which stage in development the problem occurs. E.g. Defects early on in development will affect the entire immune system. |
| − | *Myeloid cell disorders affect phagocytic function
| + | [[Lymphocytes#T cells|T cell]] deficiencies can affect both the cell-mediated and humoral response as [[Lymphocytes#T cells|T cells]] play a central role in the immune system. |
| − | *The severity of the immunodeficiency depends on at which stage in development the problem occurs
| + | |contenttitle = Content |
| − | **E.g. Defects early on in development will affect the entire immune system
| + | |contentbody =<big><b> |
| − | *[[Lymphocytes#T cells|T cell]] deficiencies can affect both the cell-mediated and humoral response as [[Lymphocytes#T cells|T cells]] play a central role in the immune system
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| − | ===Deficiencies of Innate Immunity=== | + | <categorytree mode=pages>Primary Immunodeficiency</categorytree> |
| − | [[Image:Grey Collie Syndrome.jpg|thumb|right|150px|Appearance of a puppy with Grey Collie syndrome - Copyright Michelle Tennis & Peggy Melton]]
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| − | ====Canine Cyclic Haematopoiesis====
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| − | *Also called '''Grey Collie Syndrome'''
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| − | *Autosomal recessive
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| − | *Insertion mutation in AP3B1 gene
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| − | *Diluted grey coat colour, stunted growth, poor wound healing
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| − | *Neutropenia every 2 weeks which lasts 3-4 days due to cyclic production of cells from [[Bone Marrow - Anatomy & Physiology|bone marrow]]
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| − | *Animals are prone to recurrent infections, mainly from the respiratory and gastrointestinal tract
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| − | **E.g. pyrexia, [[Diarrhoea|diarrhoea]], gingivitis and arthritis
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| − | *Puppies can be distinguished from other litter mates by the diluted grey colouring
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| − | *Affected puppies show symptoms such as fever, joint pain and eye, skin and respiratory infections from 8 weeks of age
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| − | *Affected animals rarely live beyond 2-3 years with most puppies dying within a few weeks of birth
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| − | ====Canine Leukocyte Adhesion Deficiency (CLAD)==== | + | |logo = Immature MDDC.png |
| − | *Occurs in Irish Setters
| + | }} |
| − | *Missence mutation of -Cys-36-Ser- in CD18 molecule
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| − | **CD18 is required for [[Neutrophils|neutrophil]] migration and phagocytosis
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| − | *Recurrent bacterial infection
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| − | *Neutrophilia ([[Neutrophils|neutrophils]] remain in the blood and are unable to fight infection in the tissue)
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| − | ====Bovine Leukocyte Adhesion Deficiency (BLAD)====
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| − | *Occurs in Holstein cattle
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| − | *Missence mutation of -Asp-128-Gly in CD18 molecule
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| − | *Recurrent infection, e.g. pneumonia
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| − | ===Deficiencies of Adaptive Immunity===
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| − | ====Equine Severe Combined Immune Deficiency (Equine SCID)====
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| − | *Autosomal recessive
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| − | *Occurs in 2-3% of Arabian foals
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| − | *Defect in DNA-dependent protein kinase gene
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| − | **Gene codes for a DNA repair enzyme involved in V(D)J recombination for antigen receptors of [[Lymphocytes|lymphocytes]] (e.g. Ig and TCR)
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| − | *No functional [[Lymphocytes#B cells|B cells]] or [[Lymphocytes#T cells|T cells]]
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| − | *Foals develop infections (usually around 8 weeks of age as maternal [[Immunoglobulins|antibody]] in [[Materno-Fetal Immunity - Introduction#Passive transfer via colostrum|colostrum]] wanes around this time)
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| − | *Foals usually die from bronchopneumonia
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| − | ====Canine X-Linked Severe Combined Immune Deficiency (Canine SCID)====
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| − | *Affects Basset Hounds and Corgis
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| − | *X-linked recessive defect in the gene coding for the IL-2 receptor
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| − | **IL-2 receptor is a receptor for the cytokine IL-2 which causes [[Lymphocytes#T cells|T cells]] to proliferate
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| − | *Causes lymphoid hypoplasia, stunted growth and increases the animal's susceptibility to infection
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| − | *Animal usually dies from pneumonia or sepsis as the level of maternal [[Immunoglobulins|antibody]] decreases
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| − | ====Selective [[IgA]] deficiency of German Shepherd Dogs====
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| − | *Poorly understood
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| − | *Linked to other disease syndromes such as deep pyoderma, inflammatory bowel disease, anal furunculosis and disseminated aspergillosis
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| − | *[[Immunoglobulin A|[[IgA]]]] deficiency so more susceptible to mucosal disease
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| − | ====Immunodeficiency of Weimaraners, Irish Wolfhounds and Miniature Dachshunds====
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| − | *Unknown aetiology
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| − | *Inherited defects
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| − | *Low levels of circulating [[Immunoglobulin M|IgM]] and [[Immunoglobulin G|IgG]]
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| − | *Impaired [[Neutrophils|neutrophil]] function
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| − | *Causes recurrent pyrexia and infections
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| − | **E.g. Rhinitis and bronchopneumonia in Irish Wolfhounds due to low [[Immunoglobulin A|[[IgA]]]]
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| − | **E.g. Pneumocytosis in Miniature Dachshunds due to low [[Immunoglobulin G|IgG]]
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| − | ===Laboratory Examples of Severe Combined Deficiency===
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| − | [[Image:Nude Mouse.jpg|right|thumb|150px|Athymic Nude Mouse - Armin Kübelbeck 2008]]
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| − | *Severe Combined Immune Deficiency(SCID)
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| − | **No functional [[Lymphocytes#B cells|B cells]] or [[Lymphocytes#T cells|T cells]]
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| − | *Athymic nude mice (no [[Thymus - Anatomy & Physiology|thymus]])
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| − | **No functional [[Lymphocytes#T cells|T cells]]
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| − | **Cell-mediated immunodeficiency
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| − | *Knock-out mice
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| − | **E.g. Gene coding for CD4, CD8, IL-10 removed
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| | [[Category:Immunodeficiencies]] | | [[Category:Immunodeficiencies]] |
