1/9. Recurrent haemoperitoneum in a mild von Willebrand's disease combined with a storage pool deficit.Haemoperitoneum secondary to haemorrhagic corpus luteum has been described in severe bleeding disorders such as afibrinogenaemia, type 3 von Willebrand's disease and patients under oral anticoagulation. We have studied one patient who presented three episodes of severe bleeding at ovulation, requiring surgery twice, with the diagnosis of mild von Willebrand's disease and mild storage pool deficiency. Mild von Willebrand's disease (associated with other thrombopathies or coagulopathies) should be considered in this pathology, although physicians would prefer to find a severe haemorrhagic disorder as the underlying condition in these cases.- - - - - - - - - - ranking = 1keywords = von (Clic here for more details about this article) |
2/9. Severe nose bleeding after intake of acetylsalicylic acid: von Willebrand disease type 2A. Case 9.This case report of a school boy with a history of severe and repeated episodes of epistaxis presents a short overview of the clinical and laboratory findings which lead to confirm the suspected diagnosis of von Willebrand disease (vWD). Suspicion of defective primary haemostasis should arise when unusual (because of their number or duration) mucosal bleeds appear in an otherwise normal and healthy patient. Because of its definitive inhibitory effect on platelet aggregation, acetylsalicylic acid (more than other non-steroidal anti-inflammatory drugs exerting unselective inhibition of cyclooxygenase) is a strong factor in triggering or sustaining the bleeding disorders in these patients. Among the congenital disorder of primary haemostasis, vWD is by far the most frequent one. The difficulties of laboratory diagnosis of vWD are stressed; the promises and pitfalls of new in vitro methods for measuring primary haemostasis (PFA-100 analyzer) are discussed. An accurate diagnosis of the specific type of vWD is of critical importance for correct patient management as well as for genetic counseling.- - - - - - - - - - ranking = 0.71428571428571keywords = von (Clic here for more details about this article) |
3/9. factor xiii deficiency associated with valproate treatment.PURPOSE: We present two children who developed a deficiency of factor XIII with valproate (VPA) treatment. This coagulation disorder has not been described in association with VPA treatment in children, and only very recently in one adult patient. RESULTS: Both patients showed recurrent epistaxis as major clinical sign of a combination of decreased coagulation parameters (factor xiii deficiency with thrombocytopenia and decreased von willebrand factor, respectively). A few days after reduction or withdrawal of VPA treatment, clinical symptoms disappeared, and laboratory findings were within normal range. CONCLUSIONS: VPA is known to influence the synthetic function of the liver and the number and function of megakaryocytes. Therefore an alteration of the factor XIII level by VPA is conceivable. Our case reports suggest that bleeding symptoms during VPA treatment may be caused or aggravated by a decreased factor XIII activity. A determination of factor XIII activity should be considered before surgical procedures during VPA treatment to minimize the risk of (severe) postsurgical bleeding complications.- - - - - - - - - - ranking = 0.14285714285714keywords = von (Clic here for more details about this article) |
4/9. Acquired von Willebrand disease in a patient with monoclonal gammopathy of undetermined significance.We describe a patient with acquired von Willebrand disease (vWD), who had typical presentation and clinical manifestations of acquired vWD, but correct diagnosis was delayed because of lack of awareness and early recognition. The patient was initially seen at a community hospital with persistent nasal mucosal bleeding and was transfused with an excessive amount of cryoprecipitates before appropriate hemostatic evaluation. hemostasis work-up revealed that he had classic features of acquired vWD. Further extensive evaluation revealed the patient to have monoclonal gammopathy of undetermined significance (MGUS). He was refractory to a high dose of corticosteroids and had a very transient response to desmopressin acetate (less than 4 hours) but responded well to a high dose of intravenous immunoglobulin (IVIG), which lasted at least 3 weeks with each treatment. He remained very responsive to IVIG 2 years later. His underlying MGUS has not progressed during the past 4 years.- - - - - - - - - - ranking = 0.71428571428571keywords = von (Clic here for more details about this article) |
5/9. percutaneous coronary intervention in a patient with von Willebrand's disease presenting with an acute coronary syndrome.The optimal management strategy for patients with von Willebrand's disease presenting with acute coronary syndromes is unclear. We present a clinical case of percutaneous coronary intervention following an acute coronary syndrome in a man with von Willebrand's disease. Other published case reports are reviewed. The central role of von willebrand factor in the pathophysiology of acute coronary syndrome and the possible cardioprotective effects of low levels of the protein are discussed. Practical considerations regarding hemostasis and antiplatelet therapy are addressed. Finally, recommendations for the management of patients with von Willebrand's disease presenting with acute coronary syndromes are suggested.- - - - - - - - - - ranking = 1.1428571428571keywords = von (Clic here for more details about this article) |
6/9. Acquired von Willebrand's syndrome. Therapeutic and diagnostic implications.Acquired von Willebrand's syndrome is a newly recognized bleeding diathesis thought to be caused by autoantibodies to the von willebrand factor. Acquired von Willebrand's syndrome has been reported in association with lymphoproliferative disorders and benign monoclonal gammopathies. Clinical features and laboratory abnormalities of this disease are similar to congenital von Willebrand's disease, but the optimal treatment may differ. We describe a 75-year-old man with chronic lymphocytic leukemia and recurrent epistaxis and also discuss the pathogenesis, diagnosis, and treatment of both the congenital and acquired disorders.- - - - - - - - - - ranking = 1.1428571428571keywords = von (Clic here for more details about this article) |
7/9. The use of desmopressin in children with coagulation disorders.hemophilia a, von Willebrand's disease and uremia prolong the bleeding time in affected children. Management of hemorrhage is difficult in these patients, and often requires multiple therapeutic modalities. Desmopressin, a synthetic analog of arginine vasopressin (DDAVP), decreases the bleeding time in patients with these disorders. We describe a case of a child with uremia and spontaneous epistaxis originating from the adenoid. Bleeding was controlled with administration of DDAVP and other measures. DDAVP is effective for the rapid, temporary correction of prolonged bleeding time associated with hemophilia a, von Willebrand's disease or uremia. The use of DDAVP avoids the risks associated with blood component transfusion. DDAVP may be used as a single hemostatic agent in minor surgical procedures, or in combination with other therapeutic modalities in major surgical procedures.- - - - - - - - - - ranking = 0.28571428571429keywords = von (Clic here for more details about this article) |
8/9. High-resolution analysis of von willebrand factor multimeric composition defines a new variant of type I von Willebrand disease with aberrant structure but presence of all size multimers (type IC).In Type I von Willebrand disease, the whole series of von willebrand factor (vWF) multimers is present in plasma, but all are decreased in quantity. No structural abnormality of individual multimers has been demonstrated so far in these patients. We now describe five individuals, from two unrelated families, who had this form of the disease and in whom the complex banding pattern of each vWF multimer was markedly abnormal. Inheritance was autosomal dominant and the clinical expression was mild. A bleeding history was elicited in three of the patients and included recurrent epistaxis, menometrorrhagia, and bleeding following tooth extraction. Replacement therapy had never been required. Although vWF levels in plasma were within the normal range in all of them, the ristocetin cofactor activity was decreased in four, and the bleeding time was prolonged in three. Analysis of vWF multimeric structure by agarose gel electrophoresis, including a newly developed high-resolution technique, demonstrated that the main band of each multimer was present, but a second, well-defined band always seen in normal individuals was missing in the patients. Two additional bands had altered mobility and were less well defined than in normal subjects, and a fifth, less intense band was also undetectable in the patients. Treatment with 1-deamino-8-D-arginine vasopressin (DDAVP) was assessed in two patients. It caused the circulating levels of vWF to increase and correct the bleeding time, but did not alter the structural abnormality. This study describes, therefore, a new variant form of Type I von Willebrand disease with aberrant structure of individual repeating multimers and an associated functional abnormality of vWF. In keeping with previously accepted terminology, the designation of Type IC von Willebrand disease has been adopted for this new variant.- - - - - - - - - - ranking = 1.7142857142857keywords = von (Clic here for more details about this article) |
9/9. epistaxis, von Willebrand's type bleeding diathesis and Wilms' tumour: a case report.We report a case of persistent epistaxis in a seven-month-old child with Wilms' tumour who had an associated von Willebrand's type bleeding diathesis. Correction of the bleeding diathesis to control the epistaxis required treatment of the tumour with chemotherapy.- - - - - - - - - - ranking = 0.71428571428571keywords = von (Clic here for more details about this article) |