Abstract:
von Willebrand disease (vWD) is a bleeding disorder that is predominantly
attributable to reduced levels of von Willebrand factor (vWF) activity. vWD
prevalence is 1% in the population and vWD Type 3 is very rare. vWD Type 3 is
inherited as autosomal recessive manner and accounts for less than 5% of all cases.
Individuals with vWD type 3 can have a severe internal and joint bleeding.
However, type 3 and type 2 variants are extremely difficult to manage and there is no
guarantee that haemostasis will be achieved even when plasma concentrations have
apparently been corrected into the normal range.
We report a female child aged 10 years presented to us with heavy menstrual
bleeding for three days which continued for another seven days. This episode was
after recent onset of menarche and was diagnosed as having vWD Type 3. It was
considered as a life threatening bleeding.
Immediate and long term management of this patient with a major bleed was a
daunting task.
She was treated with Intermediate purity factor viii/ factor viii (cryoprecipitate),
activated factor vii, Blood and antifibrinolytics.
Long term endometrial suppression was the key to hinder excessive bleeding during
menstruation (which could be life threatening) as she would need prophylaxis during
each episode. Selecting the correct method which suits her best was not without
difficulty.
Intrauterine use of Mirena (levonogestrel-releasing intrauterine system-LNG-IUS)
was a better option and there are very few reports if at all of its use in a similar
situation. We have used it as a novel method to suppress excessive menstrual
bleeding (long term) in this patient with von Willebrand disease type 3.
