ISSN 2398-2950      

Hemophilia

ffelis

Introduction

  • Comprises a number of different conditions in cats: Hemophilia A, Hemophilia B and Hemophilia of Maine Coons.
  • Males predominantly affected.
  • Signs: mild-moderate-severe bleeding tendency, commonly seen as hematomata on body surface or hemarthrosis.
  • Prognosis: prolonged bleeding after surgery or trauma, may be fatal.

Pathogenesis

Etiology

  • Genetic inherited disease X-linked recessive trait confirmed for Hemophilias A and B.
  • Carried by heterozygous females; affects males and homozygous females (rare).
  • Hemophilia of Maine Coons believed to be autosomal inherited condition but not clear if it is recessive or dominant or has a more complex pattern of inheritance.

Pathophysiology

  • Factor VIII = deficient in Hemophilia A.
  • Factor VIII is active in intrinsic coagulation pathway  Secondary hemostasis 01 . Together with factor IX and calcium it forms complex which activates factor X (factor X is first coagulation factor in common pathway).
  • Factor VIII, partial or absolute, deficiency    →     mild - severe hemophilia.
  • Factor IX  = deficient in Hemophilia B.
  • Factor IX is active in intrinsic coagulation pathway. Together with factor VIII and calcium, it forms a complex which activates factor X (factor X is the first coagulation factor in the common pathway).
  • Factor IX deficiency results in hemophilia which may be mild to moderate to severe.
  • Factor I (fibrinogen) ± Factor XI = deficient in Hemophilia of Maine Coons.
  • Factor XI is active in intrinsic coagulation system whilst Factor I is in final step in the common pathway.
  • There is a lack of correlation between the severity of the Factor 1 deficiency and prolongation of clotting times; this may suggest the presence of clotting times; this may suggest the presence of dysfibrinogenemia, the presence of an inhibitor to Factor 1 or that the concurrent Factor X1 deficiency complicates matters.

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.
  • Gray C, Boag A, Walton J et al (2009) Haemophilia in Maine Coon cats. Vet Rec 164 (2), 65-66 PubMed.
  • Brown R (2008) Haemophilia in Maine Coon cats. Vet Rec 163 (22), 667 PubMed.
  • Brooks M & DeWilde L (2006) Feline Factor XII Deficiency. Comp Contin Educ Pract Vet 28 (2), 148-155 VetMedResource.
  • Goree M, Catalfamo J L, Aber S et al (2005) Characterization of the mutations causing hemophilia B in 2 domestic cats. J Vet Int Med 19 (2), 200-204 PubMed.
  • Kristensen A T, Edwards M L & Devey J (2003) Potential uses of recombinant human factor VIIa in veterinary medicine. Vet Clin North Am Small Anim Pract 33 (6), 1437-1451 PubMed.
  • Maggio-Price L, Dodds W J (1993) Factor IX deficiency (hemophilia B) in a family of British shorthair cats. JAVMA 203 (12), 1702-1704 PubMed.
  • Littlewood J D & Evans R J (1990) A combined deficiency of factor VIII and contact activation defect in a family of cats. Br Vet J 146 (1), 30-35 PubMed.
  • Maddison J E, Watson A D, Eade I G et al (1990) Vitamin K-dependent multifactor coagulopathy in Devon Rex cats. JAVMA 197 (11), 1495-1457 PubMed.

Other sources of information

  • Stokol T (2005) Disorders of haemostasis. In: E. Villiers and L. Blackwood (ed.) BSAVA Manual of Canine and Feline Clinical Pathology, 2nd ed. BSAVA, Gloucester, pp 83-98.

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