Case
Study#6
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Factor
X Deficiency
- Congenital
or Acquired
- Congenital:
autosomal recessive
- Variable
bleeding tendency at any age
- Severe (<1%): umbilical stump bleeding
- Less severe: post-hemostatic challenge
- Mildly affected: bruising, menorrhagia
Heterozygotes
(1:500 incidence)
-
Usually asymptomatic
- Rarely
have bleeding thought to be 2° to either:
- Insufficient enzymatic activity of wild type
- Inhibitory or competitive effect of mutant
Homozygotes
(1:1,000,000)
- One
of the rarest inherited clotting disorders
- Patients
are usually seriously affected
- More
frequent in consanguineous marriages
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