Felis ISSN 2398-2950

Chediak-Higashi syndrome

Contributor(s): Michael Day, Severine Tasker

Introduction

  • Cause: extremely rare inherited congenital immunodeficiency disease.
  • Signs: abnormal pigmentation of hair coat and ocular fundus.
  • May be increased susceptibility to infection and an associated bleeding tendency.
  • Treatment: symptomatic, supportive only.
  • Prognosis: guarded.

Pathogenesis

Etiology

  • Autosomal recessive inheritance.

Pathophysiology

  • Abnormal lysosomes and enlarged pink or esosinophilic granules (due to fusion of normally sized granules) within granulocyte (neutrophil, eosinophil, basophil) cytoplasm.
  • May be associated with defective neutrophil chemotaxis.
  • Platelet lack dense granules that are involved in preventing bleeding.
  • Formation of enlarged melanin granules in hair shafts and eye.

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.
  • Guilford W G (1987) Primary immunodeficiency diseases of dogs and cats. Compendium on Cont Pract Vet (6), 641-648 VetMedResource.
  • Prieur D J, Collier L L, Bryan G M, Meyers K M (1979) The diagnosis of feline Chediak Higashi syndrome. Feline Prac 9, 26-32.
  • Kramer J W, Davis B C & Prieur D J (1977) The Chediak Higashi syndrome of cats. Laboratory Investigation 36 (5), 554-556 PubMed.

Other sources of information

  • Meyers K (2000) Chediak Higashi Syndrome. In: Schalm's Veterinary Haematology. Feldman B, Zinki J G & Jain N C (eds) 5th edn. Chapter 148, pp 971-975.


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