VON WILLEBRAND DISEASE: A REVIEW OF 2 CASE REPORTS. (original) (raw)
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Seminars in Thrombosis and Hemostasis, 2006
The current standard for the diagnosis and management of patients with congenital von Willebrand disease (vWD) includes bleeding times (BTs), PFA-100 closure time (PFA-CT), factor (F) VIII:coagulant activity (C), vWF:antigen (Ag), vWF:ristocetin cofactor activity (RCo), a sensitive vWF:collagen-binding activity (CB), ristocetin-induced platelet aggregation (RIPA), analysis of vWF multimers in low-and high-resolution agarose gels, and the response to desmopressin. Guidelines and recommendations for prophylaxis and treatment of bleedings in vWD patients with vWF/FVIII concentrates should be derived from analysis of the content of these concentrates and from pharmacokinetic studies in different types of vWD patients with severe type 1, 2, or 3 vWD. The vWF/FVIII concentrates should be characterized by labeling with FVIII:C, vWF:RCo, vWF:CB, and vWF multimeric pattern, which will determine their predicted efficacy and safety in prospective management studies. Because the bleeding tendency is moderate in type 2 and severe in type 3 vWD, and because the FVIII:C levels are subnormal in type 2 and very low in type 3 vWD patients, new guidelines using vWF:RCo unit dosing for the prophylaxis and treatment of bleeding episodes are proposed. Such guidelines should be stratified for the severity of bleeding, the type of surgery (either minor or major), and also for the severity and type of vWD (i.e., either type 2 or 3 vWD).
Obstetrics & Gynecology, 2009
Von Willebrand disease (VWD) is the most common inherited bleeding disorder and may affect as many as one in 100 women. The condition results from a deficiency, dysfunction, or absence of von Willebrand factor (VWF). In women, the most common symptom of VWD is menorrhagia. Of women with menorrhagia, 5-20% have been found to have previously undiagnosed VWD. Besides menorrhagia, women with VWD are more likely to experience other conditions that manifest with abnormal reproductive tract bleeding. The patient with a suspected bleeding disorder should be referred to a hemophilia treatment center or hematologist with expertise in bleeding disorders for definitive diagnosis. After diagnosis, the first choice of therapy for the management of menorrhagia in adolescents or adult females who do not desire child bearing is still hormonal contraceptives. Women who fail hormonal contraceptives, yet desire future child bearing, and women who desire pregnancy are candidates for hemostatic therapy, which is generally reserved for patients with VWF levels less than 50 international units/dL. During pregnancy, VWF levels rise, frequently obviating the need for hemostatic therapy at the time of delivery. Minor procedures can be managed with 1-desamino-8-D-arginine vasopressin, antifibrinolytic medication, or both, but major surgery or childbirth requires replacement with VWF and should be conducted in a center with available hematologists, anesthesiologists, pharmacists, and laboratory support experienced in the management of bleeding disorders. III.
Von Willebrand Disease: An Update
Journal of Blood Disorders & Transfusion, 2014
Von Willebrand disease (VWD) is a common hereditary bleeding disorder, inherited either as an autosomally dominant or recessive trait. It is caused by a quantitative and/ or qualitative defect in the Von Willebrand Factor (VWF) which plays two critical roles in hemostasis i.e., platelet adhesion and aggregation to damaged endothelium, and also as a carrier for Factor VIII (FVIII) molecule which stabilizes FVIII in the circulation. It is a highly heterogeneous disease with bleeding ranging from mild bleeding tendencies to severe life threatening hemorrhage. Patients are classified as type 1, type 2 and type 3, depending on the qualitative and quantitative defects in VWF antigen. Type 3 i.e., severe VWD has been reported to be the most prevalent subtype in Indian population due to two reasons i.e., higher degree of consanguinity in certain parts of the country and the fact that it is a hospital prevalence data and only patients with severe manifestations present themselves in the hospital. The most common bleeding symptoms in VWD reflect the characteristic defect in platelet adhesion and mucocutanous bleeding especially epistaxis, gum bleeding, menorrhagia and ecchymoses. Bleeding symptoms are of mild to moderate severity for patients with type 1 and few variants of type 2 VWD. However, life-threatening bleeding (Central nervous system, gastrointestinal bleed) can occur in type 3 and type 2 VWD patients, and rarely in type 1 VWD patients. Uncommon bleeding manifestations, such as hemarthrosis, and muscle haematomas are more common in severe deficiency, especially those who have type 3 VWD. Diagnosis, genetic counseling, carrier and antenatal diagnosis plays an important role in the comprehensive management of these cases.
Pakistan Journal of Medical and Health Sciences, 2022
Background: Women with von Willebrand disease experiences more postpartum hemorrhage or the bleeding followed by surgery than the healthy women. Aim: To determine the frequency of Von Willebrand disease among females presenting with bleeding complaints at tertiary care hospital. Study Design: Cross sectional study. Methodology: Complete Blood Count, Bleeding Time, Prothrombin time, Activated Partial Thromboplastin Time were performed on the same day. Blood sample for CBC collected in EDTA tubes and for coagulation assays in tubes containing (3.2%) trisodium citrate. Von Willebrand's factor: Ag and Factor VIII were analyzed on Sysmex automated blood coagulation analyzer CA (600) while Ristocetin cofactor assay (RiCoF) was performed on Aggregometer. The collected data was analyzed by using SPSS version 25. Chi square ware applied with P-value< 0.05 as significant. Results: Out of 80 patients, 56 patients were unmarried and 24 were married. VWD was found in 22/80 patients (27.5%). Type 3(50%) was the most frequent subtype followed by type2 (22.7%) and type 1(22.7%). In VWD, Menorrhagia (63.6%) was the most frequent symptom. APTT was prolonged in type3 VWD (50%). The mean VWF: Ag 18.5±20, VWF: RICOF 27.7±19 and factor VIII 74.9 ± 62.3 was noted in patients with VWD. Conclusion: It was concluded that VWD is the most common inherited bleeding disorder in females presented with bleeding complaints. Awareness about the disorder is the need of time in order to prevent life threatening bleeding.