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Category:Primary Immunodeficiency (view source)
Revision as of 13:40, 12 August 2010
, 13:40, 12 August 2010Created page with "*Primary immunodeficiencies may affect either the innate immune system or the adaptive immune system *..."
*Primary immunodeficiencies may affect either the [[Innate Immune System - WikiBlood|innate immune system]] or the [[Adaptive Immune System - WikiBlood|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
*[[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
===Deficiencies of Innate Immunity===
[[Image:Grey Collie Syndrome.jpg|thumb|right|150px|Appearance of a puppy with Grey Collie syndrome - Copyright Michelle Tennis & Peggy Melton]]
====Canine Cyclic Haematopoiesis====
*Also called '''Grey Collie Syndrome'''
*Autosomal recessive
*Insertion mutation in AP3B1 gene
*Diluted grey coat colour, stunted growth, poor wound healing
*Neutropenia every 2 weeks which lasts 3-4 days due to cyclic production of cells from [[Bone Marrow - Anatomy & Physiology|bone marrow]]
*Animals are prone to recurrent infections, mainly from the respiratory and gastrointestinal tract
**E.g. pyrexia, [[Diarrhoea|diarrhoea]], gingivitis and arthritis
*Puppies can be distinguished from other litter mates by the diluted grey colouring
*Affected puppies show symptoms such as fever, joint pain and eye, skin and respiratory infections from 8 weeks of age
*Affected animals rarely live beyond 2-3 years with most puppies dying within a few weeks of birth
====Canine Leukocyte Adhesion Deficiency (CLAD)====
*Occurs in Irish Setters
*Missence mutation of -Cys-36-Ser- in CD18 molecule
**CD18 is required for [[Neutrophils|neutrophil]] migration and phagocytosis
*Recurrent bacterial infection
*Neutrophilia ([[Neutrophils|neutrophils]] remain in the blood and are unable to fight infection in the tissue)
====Bovine Leukocyte Adhesion Deficiency (BLAD)====
*Occurs in Holstein cattle
*Missence mutation of -Asp-128-Gly in CD18 molecule
*Recurrent infection, e.g. pneumonia
===Deficiencies of Adaptive Immunity===
====Equine Severe Combined Immune Deficiency (Equine SCID)====
*Autosomal recessive
*Occurs in 2-3% of Arabian foals
*Defect in DNA-dependent protein kinase gene
**Gene codes for a DNA repair enzyme involved in V(D)J recombination for antigen receptors of [[Lymphocytes|lymphocytes]] (e.g. Ig and TCR)
*No functional [[Lymphocytes#B cells|B cells]] or [[Lymphocytes#T cells|T cells]]
*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)
*Foals usually die from bronchopneumonia
====Canine X-Linked Severe Combined Immune Deficiency (Canine SCID)====
*Affects Basset Hounds and Corgis
*X-linked recessive defect in the gene coding for the IL-2 receptor
**IL-2 receptor is a receptor for the cytokine IL-2 which causes [[Lymphocytes#T cells|T cells]] to proliferate
*Causes lymphoid hypoplasia, stunted growth and increases the animal's susceptibility to infection
*Animal usually dies from pneumonia or sepsis as the level of maternal [[Immunoglobulins|antibody]] decreases
====Selective [[IgA]] deficiency of German Shepherd Dogs====
*Poorly understood
*Linked to other disease syndromes such as deep pyoderma, inflammatory bowel disease, anal furunculosis and disseminated aspergillosis
*[[Immunoglobulin A|[[IgA]]]] deficiency so more susceptible to mucosal disease
====Immunodeficiency of Weimaraners, Irish Wolfhounds and Miniature Dachshunds====
*Unknown aetiology
*Inherited defects
*Low levels of circulating [[Immunoglobulin M|IgM]] and [[Immunoglobulin G|IgG]]
*Impaired [[Neutrophils|neutrophil]] function
*Causes recurrent pyrexia and infections
**E.g. Rhinitis and bronchopneumonia in Irish Wolfhounds due to low [[Immunoglobulin A|[[IgA]]]]
**E.g. Pneumocytosis in Miniature Dachshunds due to low [[Immunoglobulin G|IgG]]
===Laboratory Examples of Severe Combined Deficiency===
[[Image:Nude Mouse.jpg|right|thumb|150px|Athymic Nude Mouse - Armin Kübelbeck 2008]]
*Severe Combined Immune Deficiency(SCID)
**No functional [[Lymphocytes#B cells|B cells]] or [[Lymphocytes#T cells|T cells]]
*Athymic nude mice (no [[Thymus - Anatomy & Physiology|thymus]])
**No functional [[Lymphocytes#T cells|T cells]]
**Cell-mediated immunodeficiency
*Knock-out mice
**E.g. Gene coding for CD4, CD8, IL-10 removed
[[Category:Immunodeficiencies]]
*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
*[[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
===Deficiencies of Innate Immunity===
[[Image:Grey Collie Syndrome.jpg|thumb|right|150px|Appearance of a puppy with Grey Collie syndrome - Copyright Michelle Tennis & Peggy Melton]]
====Canine Cyclic Haematopoiesis====
*Also called '''Grey Collie Syndrome'''
*Autosomal recessive
*Insertion mutation in AP3B1 gene
*Diluted grey coat colour, stunted growth, poor wound healing
*Neutropenia every 2 weeks which lasts 3-4 days due to cyclic production of cells from [[Bone Marrow - Anatomy & Physiology|bone marrow]]
*Animals are prone to recurrent infections, mainly from the respiratory and gastrointestinal tract
**E.g. pyrexia, [[Diarrhoea|diarrhoea]], gingivitis and arthritis
*Puppies can be distinguished from other litter mates by the diluted grey colouring
*Affected puppies show symptoms such as fever, joint pain and eye, skin and respiratory infections from 8 weeks of age
*Affected animals rarely live beyond 2-3 years with most puppies dying within a few weeks of birth
====Canine Leukocyte Adhesion Deficiency (CLAD)====
*Occurs in Irish Setters
*Missence mutation of -Cys-36-Ser- in CD18 molecule
**CD18 is required for [[Neutrophils|neutrophil]] migration and phagocytosis
*Recurrent bacterial infection
*Neutrophilia ([[Neutrophils|neutrophils]] remain in the blood and are unable to fight infection in the tissue)
====Bovine Leukocyte Adhesion Deficiency (BLAD)====
*Occurs in Holstein cattle
*Missence mutation of -Asp-128-Gly in CD18 molecule
*Recurrent infection, e.g. pneumonia
===Deficiencies of Adaptive Immunity===
====Equine Severe Combined Immune Deficiency (Equine SCID)====
*Autosomal recessive
*Occurs in 2-3% of Arabian foals
*Defect in DNA-dependent protein kinase gene
**Gene codes for a DNA repair enzyme involved in V(D)J recombination for antigen receptors of [[Lymphocytes|lymphocytes]] (e.g. Ig and TCR)
*No functional [[Lymphocytes#B cells|B cells]] or [[Lymphocytes#T cells|T cells]]
*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)
*Foals usually die from bronchopneumonia
====Canine X-Linked Severe Combined Immune Deficiency (Canine SCID)====
*Affects Basset Hounds and Corgis
*X-linked recessive defect in the gene coding for the IL-2 receptor
**IL-2 receptor is a receptor for the cytokine IL-2 which causes [[Lymphocytes#T cells|T cells]] to proliferate
*Causes lymphoid hypoplasia, stunted growth and increases the animal's susceptibility to infection
*Animal usually dies from pneumonia or sepsis as the level of maternal [[Immunoglobulins|antibody]] decreases
====Selective [[IgA]] deficiency of German Shepherd Dogs====
*Poorly understood
*Linked to other disease syndromes such as deep pyoderma, inflammatory bowel disease, anal furunculosis and disseminated aspergillosis
*[[Immunoglobulin A|[[IgA]]]] deficiency so more susceptible to mucosal disease
====Immunodeficiency of Weimaraners, Irish Wolfhounds and Miniature Dachshunds====
*Unknown aetiology
*Inherited defects
*Low levels of circulating [[Immunoglobulin M|IgM]] and [[Immunoglobulin G|IgG]]
*Impaired [[Neutrophils|neutrophil]] function
*Causes recurrent pyrexia and infections
**E.g. Rhinitis and bronchopneumonia in Irish Wolfhounds due to low [[Immunoglobulin A|[[IgA]]]]
**E.g. Pneumocytosis in Miniature Dachshunds due to low [[Immunoglobulin G|IgG]]
===Laboratory Examples of Severe Combined Deficiency===
[[Image:Nude Mouse.jpg|right|thumb|150px|Athymic Nude Mouse - Armin Kübelbeck 2008]]
*Severe Combined Immune Deficiency(SCID)
**No functional [[Lymphocytes#B cells|B cells]] or [[Lymphocytes#T cells|T cells]]
*Athymic nude mice (no [[Thymus - Anatomy & Physiology|thymus]])
**No functional [[Lymphocytes#T cells|T cells]]
**Cell-mediated immunodeficiency
*Knock-out mice
**E.g. Gene coding for CD4, CD8, IL-10 removed
[[Category:Immunodeficiencies]]