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Immunology: granulocytopathy syndrome
Introduction
- Rare autosomal recessive immunodeficiency disease first reported in Irish Setter but now recognized rarely in other breeds.
- Cause: congenital defect in neutrophil function.
- Signs: recurrent severe bacterial infections in young animals.
- Diagnosis: signalment, laboratory data.
- Treatment: symptomatic, antibiotics.
- Prognosis: guarded.
Presenting signs
- Recurrent severe bacterial infections including:
- Suppurative skin lesions Skin: deep pyoderma.
- Pododermatitis Skin: pododermatitis and furunculosis.
- Gingivitis Periodontal disease.
Age predisposition
- Young - signs often seen shortly after birth.
- Mortality:
- Reduced life expectancy.
Breed/Species predisposition
- Irish Setter Irish Setter.
- Doberman Pinscher Dobermann.
- Weimaraner Weimaraner.
- Brittany spaniels Brittany.
Pathogenesis
Etiology
- Autosomal recessive in Irish Setter.
- Mode of inheritance not established in other breeds.
Pathophysiology
- Impaired immune-response → recurrent bacterial infections.
- Neutrophils defective in ability to adhere to bacteria (Irish Setter) as a result of lack of two surface glycoproteins Mol1 and LFA-1.
- Neutrophils defective in ability to kill phagocytosed bacteria (Doberman Pinscher).
- Neutrophils defective in oxidative metabolism (Weimeraners).
- Complement deficiency reported in a colony of Brittany spaniels Immunology: C3 deficiency.
- Normal humoral and cell-mediated immune responses.
Timecourse
- Weeks.
Diagnosis
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Treatment
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Prevention
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Outcomes
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Further Reading
Publications
Refereed papers
- Recent references from PubMed and VetMedResource.
- Couto C G et al (1989) In vitro immunologic features of Weimeraner dogs with neutrophil abnormalities and recurrent infections. Vet Immunol 23 (1-2), 103-12 PubMed.
- Breitschwerdt E B et al (1987) Rhinitis, pneumonia and defective neutrophil function in the Doberman Pinscher. AJVR 48 (7), 1054-62 PubMed.
- Giger U, Boxer L A, Simpson P J, Lucchesi B R & Todd R F (1987) Deficiency of leukocyte glycoproteins Mo1, LFA-1 and Leu M5 in a dog with recurrent bacterial infections. Blood 69 (6), 1622-30 PubMed.
- Guilford W G (1987) Primary immunodeficiency diseases of dogs and cats. Comp Cont Educ 9 (6), 641-650 VetMedResource.
- Blum J R, Cork L C & Morris J M et al (1985) The clinical manifestations of a genetically determined deficiency of C3 in the dog. Clin Immunol Immunopathol 34 (3), 304-15 PubMed.
- Renshaw H W & Davis W C (1979) Canine granulocytopathy syndrome: an inherited disorder of leukocyte function. Am J Pathol 95 (3), 731-744 PubMed.
- Renshaw H W, Chatburn C, Bryan G, Bartsch R C & Davis W (1975) Canine granulocytopathy syndrome- neutrophil dysfunction in a dog with recurrent infections. JAVMA 166 (5), 443-447 PubMed.
Other sources of information
- Felsberg, P J (1992)Primary immunodeficiencies.In:Current Veterinary Therapy IX: Small Animal Practice.Eds: Kirk R W & Bonagura J D. Philadelphia: W B Saunders. pp 448-453.
- Thompson JP (1995).Immunologic diseases.In:Textbook of Veterinary Internal Medicine.4th edn. Eds: S J Ettinger & E C Feldman. Philadelphia: WB Saunders. pp 2002-2029.