ISSN 2398-2969      

Immunology: granulocytopathy syndrome

icanis
Contributor(s):

Richard Squires


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.

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.

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