ISSN 2398-2969      

Cyclic hematopoiesis

icanis
Contributor(s):

Kyle Braund

Simon Tappin

Synonym(s): Canine cyclic neutropenia


Introduction

  • Rare condition of Collie.
  • Cause: genetic (autosomal recessive) mutation in the AP3B1 gene leading to disrupted activity of neutrophil elastase and reduced activity of the granulocyte colony-stimulating factor signalling pathway.
  • Signs: born with silver grey coat and pale colored nose.
  • Leads to cyclic neutropenia on a 10-12 day cycle.
  • Diagnosis: breed, coat and nose color, clinical signs.
  • Treatment: systemic treatment of secondary infections, bone marrow stimulation with lithium carbonate and recombinant human granulocyte colony-stimulating factor, bone marrow transplant, eventual euthanasia.
  • Prognosis: animals die within 2-3 years of birth.

Pathogenesis

Etiology

  • Caused by an autosomal recessive missense mutation in the canine AP3B1 gene. This gene encodes for the beta subunit of the neutrophil adaptor protein complex 3 leading to reduced neturophil elastase enzymatic activity. This causes a defect in the granulocyte colony-stimulating factor signalling pathway leading to disease of stem cells in affected dogs.

Pathophysiology

  • Genetically determined.
  • Coat color linked.
  • Recurrent neutropenia (10-12 day intervals) allows significant recurring infection (each episode lasting 3-5 days).
  • Predisposed to a bacterial disease during neutropenic phases and to amyloidosis secondary to chronic bacterial infections.
  • Fatal hepatic or renal amyloidosis Liver: amyloidosis Amyloidosis supervenes within 2 years if puppies are kept alive.

Timecourse

  • Only 2% of affected dogs survive to 1 year of age.

Diagnosis

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Treatment

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Outcomes

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Further Reading

Publications

Refereed papers

  • Recent references from PubMed and VetMedResource.
  • Meng R et al (2010) Neutrophil elastase-processing defect in cyclic hematopoietic dogs. Exp Hematol 28 (2), 104-115 PubMed.
  • Yanay O et al (2003) Treatment of canine cyclic neutropenia by lentivirus-mediated G-CSF delivery. Blood 102 (6), 2046-2052 PubMed.
  • Brabb T et al (1995) Pancreatic adenocarcinoma in two Grey Collie dogs with cyclic hematopoiesisLab Anim Sci 45 (4), 357-362 PubMed.
  • Moazed T C et al (1990) Subcutaneous abscess due to Salmonella adelaide in a Grey Collie with cyclic hematopoiesisLab Anim Sci 40 (6), 639-641 PubMed.
  • Lothrop C D et al (1988) Correction of canine cyclic hematopoiesis with recombinant human granulocyte colony-stimulating factor. Blood 72 (4), 1324-1328 PubMed.
  • Yang T (1987) Gray Collie syndrome. JAVMA 191 (4), 390-1 PubMed.
  • Hammond W P & Dale D D (1980) Lithium therapy of canine cyclic hematopoiesis. Blood 55 (1), 26-8 PubMed.

Other sources of information

  • Day M J & Schultz R D (2011)Veterinary Immunology Principles and Practice.Manson Publishing, pp 185-186.

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