Causes and risk factors
Von Willebrand disease is an inherited genetic disorder. Deficiency of von willebrand factor causes this disease. There are three major types of VWD- Type 1 is mildest and commonest form. It is characterized by low levels of von Willebrand factor and may also have low levels of factor VIII. In Type 2 Von Willebrand factor is present but doesn’t work well. In Type 3 Von Willebrand factor is absent and factor VIII levels are low. Type 3 is the rarest and most serious form of VWD.
Clinical presentation
Patient with VWD presents with recurrent nosebleeds, gum bleed. Women experience profuse menstrual flow. There is excessive bleeding following any dental procedure, excessive bleeding even from minor injuries, easy bruising. Skin rash is observed. Internal bleeding, bleeding in joints is common. Blood in urine or stools is also experienced.
Investigation
Medical history by the patient and Clinical examination by the doctor helps in diagnosis. Routine blood test is recommended. Platelet count is estimated.BT, CT is advised. Special blood test like Factor VIII levels, Platelet aggregation test, Ristocetin cofactor test[which shows how well the von Willebrand factor works in the clotting process], VWF antigen test, platelet function test [PFA] are carried out to confirm the diagnosis.
Treatment
Medications to raise von Willebrand factor level and reduce the chances of bleeding are given intravenously or through a nasal spray. Blood replacement therapy of blood-clotting factors containing von Willebrand factor is required. Anti-fibrinolytic or clot-stabilizing medications are prescribed before carrying out any kind of major or minor surgical procedure on the patient to control the bleeding. Profuse menstrual flow in women can be controlled with the help of oral contraceptive pills. Fibrin sealants [glue like substances] are placed directly on a cut to stop bleeding which also help in managing VWD.
