Disease overview

Hemophilia B is a deficiency in Factor IX (a secondary hemostatic disease), which leads to a failure of platelet plug stabilization during the coagulation/clotting pathway.

Gene name

F9

Inheritance

X-Linked recessive

Signs and symptoms

Hemophilia B can cause mild to severe bleeding, with possible spontaneous bleeding depending on the degree of Factor IX deficiency. Clotting conditions are typically recognized during trauma or preparation for surgery.

How is it diagnosed

Secondary hemostatic diseases are diagnosed with a range of blood tests that determine blood clotting times.

Breeds at higher risk

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Citations

Brinkhous KM, Hedner U, Garris JB, Diness V, Read MS. Effect of recombinant factor VIIa on the hemostatic defect in dogs with hemophilia A, hemophilia B, and von Willebrand disease. Proc Natl Acad Sci U S A. 1989 Feb;86(4):1382-6. doi: 10.1073/pnas.86.4.1382. PMID: 2784006; PMCID: PMC286694.

Mauser AE, Whitlark J, Whitney KM, Lothrop CD Jr. A deletion mutation causes hemophilia B in Lhasa Apso dogs. Blood. 1996 Nov 1;88(9):3451-5. PMID: 8896410.

Brooks MB, Gu W, Ray K. Complete deletion of factor IX gene and inhibition of factor IX activity in a labrador retriever with hemophilia B. J Am Vet Med Assoc. 1997 Dec 1;211(11):1418-21. PMID: 9394892.

Mischke R, Kühnlein P, Kehl A, Langbein-Detsch I, Steudle F, Schmid A, Dandekar T, Czwalinna A, Müller E. G244E in the canine factor IX gene leads to severe haemophilia B in Rhodesian Ridgebacks. Vet J. 2011 Jan;187(1):113-8. doi: 10.1016/j.tvjl.2010.01.017. Epub 2010 Mar 29. PMID: 20303304.

Brenig B, Steingräber L, Shan S, Xu F, Hirschfeld M, Andag R, Spengeler M, Dietschi E, Mischke R, Leeb T. Christmas disease in a Hovawart family resembling human hemophilia B Leyden is caused by a single nucleotide deletion in a highly conserved transcription factor binding site of the F9 gene promoter. Haematologica. 2019 Nov;104(11):2307-2313. doi: 10.3324/haematol.2018.215426. Epub 2019 Mar 7. PMID: 30846504; PMCID: PMC6821609.

Kuder H, Sandzhieva-Vuzzo L, Kehl A, Rappaport JM, Müller E, Giger U. A Single Base Insertion in F9 Causing Hemophilia B in a Family of Newfoundland-Parti Standard Poodle Hybrid Dogs. Genes (Basel). 2021 Sep 24;12(10):1491. doi: 10.3390/genes12101491. PMID: 34680886; PMCID: PMC8535623.

Gu W, Brooks M, Catalfamo J, Ray J, Ray K. Two distinct mutations cause severe hemophilia B in two unrelated canine pedigrees. Thromb Haemost. 1999 Oct;82(4):1270-5. PMID: 10544912.

Disclaimer

Disease diagnosis and treatment should always be performed by a veterinarian. The following information is for educational purposes only.