Cases reported "Epistaxis"

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1/12. Glanzmann's thrombasthenia in a Melanesian.

    A case of Glanzmann's thrombasthenia is documented in a Melanesian child, and the diagnosis and treatment of this disorder are discussed. This is the third inherited bleeding disorder to be reported in papua new guinea, haemophilia A (factor viii deficiency) and factor xiii deficiency having been described previously.
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ranking = 1
keywords = haemophilia
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2/12. 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.
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ranking = 8.8366623030449
keywords = von
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3/12. An unusual cause of epistaxis: a haemophilic pseudotumour in a non-haemophiliac, arising in a paranasal sinus.

    Most cases of epistaxis are due to simple causes and are easily treated on an out-patient basis. However, there are some cases where the origin of bleeding is not obvious or arises from an unusual pathological source. The authors describe a case of epistaxis due to a mass in the maxillary antrum that when biopsied showed the histological appearances of a haemophilic pseudotumour. The patient was anticoagulated on warfarin for a cardiac valve replacement and this was thought to be the cause of the ongoing haemorrhage necessary for development of the pseudotumour. Even in haemophiliacs, pseudotumours are rare and we believe this case is unique in that the patient is a non-haemophiliac. The epistaxis was eventually controlled by external beam radiotherapy to the pseudotumour. The management of this case is outlined as well as a review of the literature on haemophilic pseudotumour.
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ranking = 6
keywords = haemophilia
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4/12. 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.
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ranking = 6.3119016450321
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5/12. 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.
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ranking = 1.2623803290064
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6/12. 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.
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ranking = 6.3119016450321
keywords = von
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7/12. Use of recombinant factor viia in the management of severe bleeding episodes in patients with bernard-soulier syndrome.

    bernard-soulier syndrome (BSS) is a rare congenital platelet disorder characterized by defective platelet adhesion and manifested by spontaneous and often profuse bleeding. Recombinant factor viia (rFVIIa) is a haemostatic agent licensed for the treatment of bleeding episodes in patients with haemophilia and inhibitors, which may represent a low-risk alternative to existing therapies in the management of patients with BSS. Here, we describe the use of rFVIIa for the treatment of three severe bleeding episodes in two patients with BSS. Data were extracted by automated searching of the international, internet-based registry http://www.haemostasis.com . Patient 1, a 24-year-old woman, was admitted with severe epistaxis and hypotension. The diagnosis of BSS was confirmed by macrothrombocytopenia, absence of ristocetin-induced platelet agglutination (RIPA) and absence of glycoprotein (GP) Ibalpha and IX on the platelet surface. Epsilon aminocaproic acid (EACA; two 50-mg/kg doses), packed red blood cells (PRBCs, 2 U) and platelets (30 U) failed to control the bleeding and, after 13 h, three bolus doses of rFVIIa (90 microg/kg body weight) and a third dose of EACA were administered; bleeding stopped after the third dose of rFVIIa. Patient 2, a 15-year-old girl, initially presented with severe menorrhagia. A lack of RIPA and severe deficiency of GPIbalpha on the platelet surface confirmed the diagnosis of BSS. EACA and fresh-frozen plasma did not control the haemorrhage, but two bolus doses of rFVIIa (98 microg/kg body weight) resulted in a marked decrease in bleeding. On second admission, patient 2 had severe epistaxis and mild menorrhagia. Two rFVIIa doses (98 and 122.5 microg/kg body weight) were given, and the bleeding stopped. No adverse events were reported in these cases. These three admissions highlight the potential of rFVIIa for the treatment of severe bleeds in patients with BSS.
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ranking = 1
keywords = haemophilia
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8/12. 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.
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ranking = 10.099042632051
keywords = von
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9/12. 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.
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ranking = 10.099042632051
keywords = von
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10/12. 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.
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ranking = 2.5247606580128
keywords = von
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