Management of Type 2B von Willebrand Disease during Pregnancy
| العنوان: | Management of Type 2B von Willebrand Disease during Pregnancy |
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| المؤلفون: | David McLaughlin, Ron Kerr |
| المصدر: | Acta Haematologica. 137:89-92 |
| بيانات النشر: | S. Karger AG, 2017. |
| سنة النشر: | 2017 |
| مصطلحات موضوعية: | Adult, Male, Pediatrics, medicine.medical_specialty, Mutation, Missense, von Willebrand Disease, Type 2, 030204 cardiovascular system & hematology, 03 medical and health sciences, 0302 clinical medicine, Full recovery, Von Willebrand factor, Pregnancy, hemic and lymphatic diseases, von Willebrand Factor, Von Willebrand disease, medicine, Humans, Missense mutation, Platelet, Labor, Obstetric, biology, business.industry, Pregnancy Complications, Hematologic, First pregnancy, Hematology, General Medicine, medicine.disease, Amino Acid Substitution, biology.protein, Female, business, Tranexamic acid, circulatory and respiratory physiology, 030215 immunology, medicine.drug |
| الوصف: | Type 2B von Willebrand disease is a rare bleeding condition resulting in thrombocytopenia and a reduction in large VWF multimers. It usually has an autosomal dominant pattern of inheritance. We report the management of a patient with type 2B von Willebrand disease, whose diagnosis was confirmed by demonstration of a R1306W mutation, through her first pregnancy. The patient's von Willebrand factor (VWF) antigen and VWF ristocetin cofactor levels rose throughout pregnancy, with an associated drop in the platelet count. The patient was successfully managed through labour to a surgical delivery with VWF concentrate, platelet transfusions and tranexamic acid. The patient delivered a male baby who was found to have inherited type 2B von Willebrand disease and had a significant cephalhaematoma at delivery. The baby was managed with VWF concentrate and platelet transfusions and made a full recovery. There is a lack of evidence to guide the best management of pregnant patients with type 2B von Willebrand disease. We adopted a pragmatic management plan, in keeping with other published case reports. To the best of our knowledge, this is the first case report in which the child was found to have inherited type 2B von Willebrand disease and encountered bleeding problems, making this case unique amongst the published literature. |
| تدمد: | 1421-9662 0001-5792 |
| DOI: | 10.1159/000453389 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::00b8fca5a4cbf8ba76399fd7ae4a230e https://doi.org/10.1159/000453389 |
| Rights: | CLOSED |
| رقم الانضمام: | edsair.doi.dedup.....00b8fca5a4cbf8ba76399fd7ae4a230e |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 14219662 00015792 |
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| DOI: | 10.1159/000453389 |