PB1849 WHEN DO WE HAVE TO SUSPECT AND DIAGNOSE VON WILLEBRAND DISEASE?

Abstract

Background:Although von Willebrand disease (vWD) is the most common hereditary coagulation disorder, most of patients do not pay attention to their daily lives. While it is easy to treat already diagnosed vWD patients, it is also a characteristic of the disease that it is not easy to suspect and diagnose. The patient does not tell the doctor easy bruise as a major symptom because he (or she) does not know that this may have been caused by coagulation disease. It is very important to ask the patient whether he suffers from ’easy bruise or intractable nosebleed?’, ’ever suffered from poor stop‐bleeding on the injection site?’ or ’a family history of bleeding disorders?’ to determine the pressure time (10 minutes or more) to stop the injection site bleeding in cases of invasive procedure such as vaccination, blood collection or injection even when the patient is diagnosed and treated with other diseases. Menorrhagia or hypermenorrhea, delivery history and family history of coagulation disorder is hard to get medical history without specially asking. The reason why we are difficult to suspect and diagnose vWD: Many think easy bruise is not a disease. No one considered a repeat intractable nosebleed after stop as a result of basically coagulation defect. It seems to accept menorrhagia or hypermenorrhea as a mother's similarity and to solve only by gynecological treatment. Most doctors fail to treat iron deficient anemia because they do not pay attention for etiology. Do not recognize repeat test may be necessary because exercise and stress will make false positive result of vWF.Aims:Objective of this study was to examine the icidence and clinical features of VWD among the patients with easy bruising in the setting of primary care clinic.Methods:The screening test and the primary specific test for vWD (FVIIIc, vWF:RiCoF and vWF:Ag) and iron deficiency anemia (IDA) were done in 182 patients with bleeding symptoms (menorrhagia, easy bruising, etc.) among the patients who visited one private physician's clinic (Han's Clinic) for 4.5 years.Results:Twenty‐one patients were confirmed as vWD (vWF:RCo or vWF:Ag value was less than 30 or vWF:RCo/vWF:Ag < 0.6) and 23 had vWF values less than 50. All except two were female. Median age was 34.7 (13‐71) years. Six of 21 patients had a family history. Most of them had iron‐deficiency anemia of moderate to severe. The main symptom was easy bruise in 16, anemia in 10, menorrhagia in 7, hypermenorrhea in 5, easy epistaxis in 2 and dizziness in 1. Iron deficiency anemia was confirmed in 8 of 21 patients. Four patients received prophylactic therapy with Minirin® effectively and 1 had treated with Immunate® for tooth extraction and 50 days’ menstruation. The menstrual period lasting for one month was reduced to normal with DDAVP inhalation and vWF infusion could stop bleeding soon after tooth extraction.Summary/Conclusion:Because accidents or emergency surgery can happen in life time to anyone, early suspicion and diagnosis will save life and reduce sequelae.