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HAEMOPHILIA C

haemophilia C (also known as plasma thromboplastin antecedent (PTA)


deficiency or Rosenthal syndrome) is a mild form of haemophilia affecting
both sexes, due to factor XI deficiency. It is the fourth most
common coagulation disorder after von Willebrand's disease and haemophilia
A and B.

symptoms
In terms of the signs/symptoms of haemophilia C, unlike individuals
with Haemophilia A and B, people affected by it are not ones to bleed
spontaneously. In these cases, haemorrhages tend to happen after a major
surgery or injury. However, people affected with haemophilia C might
experience symptoms closely related to those of other forms of haemophilia
such as the following:

 Oral bleeding.

 Nosebleeds

 Blood in the urine

 Post-partum bleeding (20% of cases)

 Tonsils (bleeding)

Diagnosis
haemophilia C (factor XI deficiency) is centered on prolonged activated partial
thromboplastin time (aPTT).One will find that the factor XI has decreased in
the individuals body. In terms of differential diagnosis one must
consider: haemophilia A, haemophilia B, lupus
anticoagulant and heparin contamination. The prolongation of the activated
partial thromboplastin time should completely correct with a 1:1 mixing
study with normal plasma if haemophilia C is present; in contrast, if a lupus
anticoagulant is present as the cause of a prolonged a PTT
Treatment
In terms of haemophilia C
medication cyklokapron is often used for both
treatment after an incident of bleeding and as a
preventive measure to avoid excessive bleeding
during oral surgery.
Treatment is usually not necessary, except in relation
to operations, leading to many of those having the
condition not being aware of it. In these cases, fresh
frozen plasma or recombinant factor XI may be
used, but only if necessary.
The afflicted may often suffer nosebleeds, while
females can experience unusual menstrual bleeding
which can be avoided by taking birth control such
as: IUDs and oral or injected contraceptives to
increase coagulation ability by adjusting hormones
to levels similar to pregnancy.

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