Prophylaxis in von Willebrand disease. Case report Presented by Aziz Eghbali. MD, Pediatric Hematologist & Oncologist, medical university of Arak.Arak.Iran.

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Presentation transcript:

Prophylaxis in von Willebrand disease. Case report Presented by Aziz Eghbali. MD, Pediatric Hematologist & Oncologist, medical university of Arak.Arak.Iran

Von Willebrand disease (VWD) Von Willebrand disease (VWD), the most common hereditary bleeding disorder, is divided into three types depending on the quantitative (type 1 and 3) or qualitative (type 2) abnormality of von Willebrand factor (VWF)

Von Willebrand disease (VWD) About 70–80% of VWD patients can be treated with the synthetic product desmopressin, while the others necessitate factor VIII/VWF concentrates. In addition to the treatment of bleeding episodes, therapeutic regimens include short- or long-term prophylaxis.

Prophylaxis in von Willebrand disease Short-term prophylaxis is usually performed to prevent excessive bleeding after surgery or invasive procedures, while long-term prophylaxis may be needed to control recurrent mucosal and joint bleeding complicating the more severe forms of VWD.

Long-term prophylaxis in von Willebrand disease Patients with more severe forms of VWD may have recurrent episodes of mucocutaneous bleeding and also hemarthrosis and hematomas and thus are candidate for long- term secondary prophylaxis. However, whereas prophylaxis in hemophilia has become widespread in recent years, this mode of treatment appears to be practiced only occasionally in VWD patients.

Case Report

An 11-year-old girl, known case of Type III von Willebrand Disease (VWD) had been referred because of recurrent epistaxis and easy bruising,for the first time, when she was 2 years old.

Case Report since that time until 3 years ago, she had been receiving plasma-derived factor VIII and from 3 years ago she has been receiving Humate-P, (VWF/FVIII concentrate), according to her demand.

Case Report Once, She had been referred for upper gastrointestinal bleeding 9 months ago with Hb: 6.5 and sever nodularity in gastric anter zone in endoscopy. Because of that, she had been admitted and had been transfused packed cell and had received Humate-p (60 unit / kg /dose) every 12 hours for 2 weeks.

Case Report Similarly, admitted with upper gastrointestinal bleeding and Hb: 6 about 2months later which was medicated by packed cell and Humate-p for 2 weeks.

Case Report Gastric mucosal biopsy showed no H-pylory infection. She discharged with PPI. She admitted again after 45 days with upper gastrointestinal bleeding and Hb: 4.5 in PICU and transfused packed cell and also received Humate-p for 2 weeks.

Case Report From last admission,about 5 months ago, she has been using Humate-p 3 times a week with dosage of 40 unit / kg / dose until now. Our follow up has shown no drop in Hb and no upper gastrointestinal bleeding.