Leukocyte Adhesion Deficiency, Type III

Other Names: Canine leukocyte adhesion deficiency, Leukocyte/Platelet adhesion defect, CLAD, LAD-III
Affected Genes: FERMT3
Inheritance: Autosomal Recessive
Mutation: chr18:52835932-52835933 (canFam3): 12 bp insertion (ins AAGACGGCTGCC)

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Common Symptoms

Leukocyte adhesion deficiency type III is an inherited blood disorder affecting dogs. Affected dogs have abnormal platelet and white blood cell activity resulting in abnormal blood clotting and immune system function. Dogs may present with lameness, prolonged bleeding and recurrent, chronic infections especially of the skin and gums and often accompanied by fever. Other symptoms include persistently elevated white blood cell counts (leukocytosis). Dogs can live for years with this condition although they are susceptible to life-threatening bleeding with an accidental injury or any surgical procedure. Veterinarians performing surgery on known affected dogs should have ready access to blood banked for transfusions.


Breed-Specific Information for the White Shepherd Dog

The White Shepherd Dog is included as a breed susceptible to leukocyte adhesion deficiency type III because of its close relatedness to the German Shepherd Dog breed, which is known to develop this disease due to Mutation of the FERMT3 gene. The frequency of the causal mutation in the general White Shepherd Dog population is unknown.


Testing Tips

Genetic testing of the FERMT3 gene in White Shepherd Dogs will reliably determine whether a dog is a genetic Carrier of leukocyte adhesion deficiency type III. Leukocyte adhesion deficiency type III is inherited in an Autosomal Recessive manner in dogs meaning that they must receive two copies of the mutated gene (one from each parent) to develop the disease. In general, carrier dogs do not have features of the disease but when bred with another carrier of the same Mutation, there is a risk of having affected pups. Each pup that is born to this pairing has a 25% chance of inheriting the disease and a 50% chance of inheriting one copy and being a carrier of the FERMT3 gene mutation. Reliable genetic testing is important for determining breeding practices. In order to eliminate this mutation from breeding lines and to avoid the potential of producing affected pups, breeding of known carriers to each other is not recommended. White Shepherd Dogs that are not carriers of the mutation have no increased risk of having affected pups.


There may be other causes of this condition in dogs and a normal result does not exclude a different mutation in this gene or any other gene that may result in a similar genetic disease or trait.


References

  • Boudreaux MK, Wardrop KJ, Kiklevich V, Felsburg P, Snekvik K. A mutation in the canine Kindlin-3 gene associated with increased bleeding risk and susceptibility to infections. Thromb Haemost. 2010 Feb; 103(2):475-7. [PubMed: 20126836]