Chediak-Higashi syndrome

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Sections available in full article Introduction, Presenting signs, Age predisposition, Breed predisposition, Pathogenesis, Etiology, Pathophysiology, Diagnosis, Client history, Clinical signs, Diagnostic investigation, Confirmation of diagnosis, Histopathology findings, Treatment, Initial symptomatic treatment, Prevention, Group eradication, Sequelae, Prognosis, Sources, Publications, Vetstream contributor(s),
Contributors Prof Michael Day BSc BVMS(Hons) PhD DSc DiplECVP FASM FRCPath FRCVS
Dr Severine Tasker BSc BVSc DSAM CertSAM DipECVIM-ca MRCVS

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.

Diagnosis

Clinical signs

  • Increased susceptibility to pyogenic infections.
  • Hypopigmentation: red (cf normal yellow-green retinal color) fundic light reflections and light blue smoke color to hair shafts.
  • Blepharospasm/photophobia.
  • Early cataracts  Cataract  .
  • Bleeding tendency  Hemostatic disorders: inherited  ; defect of primary hemostasis so usually see mulitple minor bleeds from mucosal surface and in the skin etc.

Sequelae

Prognosis

  • Guarded - severely affected individuals have poor life expectancy.

Sources

Publications

Refereed papers

  • Recent references from PubMed.
  • Kramer J W, Davis B C & Prieur D J (1997)The Chediak Higashi syndrome of cats.Laboratory Investigation36, 554-556. (Original report of the syndrome in cats.)
  • Guilford W G (1987)Primary immunodeficiency diseases of dogs and cats.Compendium on Cont Pract Vet9, 641-645. (Useful review of feline immunodeficiency.)
  • Prieur D J, Collier L L, Bryan G M, Meyers K M (1979)The diagnosis of feline Chediak Higashi syndrome.Feline Prac9, 26-32.

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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