Abstract :
Type 1 Von Willebrand disease (VWD) is the most common type of VWD due to partial quantitative deficiency of Von Willebrand factor (VWF) which is essential for hemostasis. It is inherited in an autosomal dominant pattern in most cases, but variable penetrance has also been observed in certain cases. In the present report, we report a case of 11-year-old admitted a tertiary hospital with complaints of excessive vaginal bleeding (menorrhagia), and fatigue since the past 12 days. At the age of 10 years, there was excessive bleeding from the injured site in the knee following a fall on the ground. Blood investigations revealed anemia, a decrease in Von Willebrand antigen, and collagen binding assay. USG reports showed polycystic morphology of the left ovary with thickened endometrium. There is no complete cure for this condition. Mild bleeding episodes during the menstrual cycle can be managed using oral contraceptive pills. Desmopressin has been proven to be useful in mild cases especially to achieve normal levels before medical procedures or surgery but not in severe forms of Type 1 VWD. In severe forms of Type 1 VWD, VWF concentrates have been tried.
Keyword :
Willebrand, Von Willebrand factor