von Willebrand factor in myeloproliferative disorders

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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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Platelet von Willebrand factor in inherited and acquired bleeding disorders.

The majority of plasma proteins involved in the mechanisms of hemostasis are loosely adsorbed on the platelet surface. A few of them, however, can also be measured in washed platelets, in which they are contained in the intracellular organelles called a granules. The main proteins involved in blood coagulation concentrated in platelets are von Willebrand factor (vWF), fibrinogen, and factor V. ...

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Acquired von Willebrand factor abnormalities in myeloproliferative disorders and other hematologic diseases: a retrospective analysis by a single institution.

BACKGROUND AND OBJECTIVES Acquired von Willebrand syndrome (AVWS) is a rare acquired disorder. In most cases it is associated with lymphoproliferative disorders and monoclonal gammopathies, while less frequently myeloproliferative disorders (MPD) are involved. Although bleeding is the most important symptom, thrombotic complications have also been observed in cases associated with MPD. Our aim ...

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Zebrafish von Willebrand factor.

von Willebrand factor (vWF) is a large protein involved in primary hemostasis. A dysfunction in this protein or an insufficient production of the protein leads to improper platelet adhesion/aggregation, resulting in a bleeding phenotype known as von Willebrand disease (vWD). To gain a better understanding of vWF interactions in vivo, the use of zebrafish as a model is ideal because of the trans...

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Decreased von Willebrand factor protease activity associated with thrombocytopenic disorders.

Recent studies investigating thrombotic thrombocytopenic purpura (TTP) have implicated abnormal plasma von Willebrand factor (vWF)-cleaving metalloprotease activity in this disorder. It has been proposed that a metalloprotease cleaves unusually large (UL) multimers of vWF, which enter the circulation from the endothelium. Abnormal metalloprotease activity could result in ULvWF, which could part...

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ژورنال

عنوان ژورنال: Blood & Vessel

سال: 1980

ISSN: 0386-9717,1884-2372

DOI: 10.2491/jjsth1970.11.21