7. Diagnosis and management of acquired hemophilia and acquired von Willebrand disease

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Subspecialty Clinics: Hematology Acquired von Willebrand Disease

Address reprint requests and correspondence to Rajiv K. Pruthi, MD, Division of Hematology, Mayo Clinic, 200 First St SW, Rochester, MN 55905 (e-mail: [email protected]). Acquired von Willebrand disease (AvWD) is a relatively rare acquired bleeding disorder that usually occurs in elderly patients, in whom its recognition may be delayed. Patients usually present predominantly with mucocutane...

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Acquired hemophilia A and von Willebrand syndrome in a patient with late-onset systemic lupus erythematosus

Acquired hemophilia A (AHA) and acquired von Willebrand Syndrome (AVWS) are both rare bleeding disorders that can be associated with lymphoproliferative or autoimmune diseases. AHA is uniformly caused by inhibitory autoantibodies against coagulation factor VIII (FVIII), while the pathophysiology of AVWS comprises several distinct mechanisms, including reduced synthesis, accelerated clearance, o...

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High-dose intravenous gammaglobulin therapy for acquired von Willebrand disease.

Patients with acquired von Willebrand disease may present with severe bleeding, which is usually difficult to manage. Adequate haemostasis in acquired von Willebrand disease may be achieved with the infusion of factor VIII/von Willebrand factor concentrates or with the administration of desmopressin. We report a case of acquired von Willebrand disease with severe postoperative bleeding, respond...

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Acquired von Willebrand syndrome in aortic stenosis.

BACKGROUND Aortic-valve stenosis can be complicated by bleeding that is associated with acquired type 2A von Willebrand syndrome. However, the prevalence and cause of the hemostatic abnormality in aortic stenosis are unknown. METHODS We enrolled 50 consecutive patients with aortic stenosis, who completed a standardized screening questionnaire to detect a history of bleeding. Forty-two patient...

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Acquired von Willebrand syndrome in myeloproliferative disorder

We present a woman (age: 57 years) with an excessive bleeding episode under acetylsalicylic acid after bone marrow puncture due to an acquired von Willebrand syndrome (avWS) in the context of a myeloproliferative disorder. The laboratory features showed a high platelet concentration and a qualitative defect of von Willebrand factor (vWF) with a low normal vWF ristocetin cofactor activity, a nor...

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ژورنال

عنوان ژورنال: Japanese Journal of Thrombosis and Hemostasis

سال: 2007

ISSN: 1880-8808,0915-7441

DOI: 10.2491/jjsth.18.575