Profound alterations of the multimeric structure of von Willebrand factor in a patient with malignant lymphoma

C. Tran-Thang, P. M. Mannucci, P. Schneider, A. Federici, F. Bachmann

Research output: Contribution to journalArticlepeer-review

Abstract

Laboratory investigation of an acquired haemorrhagic diathesis in a 63-year-old man with malignant lymphoma revealed the classical haemostatic defects found in von Willebrand's disease (vWD). In addition, SDS-agarose gel electrophoresis demonstrated alterations of the von Willebrand factor (vWF) multimeric structure. A profound defect of large and intermediate size multimers was observed which was different from those seen in variants of congenital vWD. In vitro, weak inhibitory activity against factor VIII procoagulant activity and ristocetin cofactor activity was present in the patient's plasma. When patient's plasma was incubated with normal plasma, followed by centrifugation, vWF antigen (vWF:Ag) was precipitated. In vivo, after transfusion of cryoprecipitate, there was rapid plasma clearance of vWF:Ag and ristocetin cofactor and of FVIII coagulant activities.

Original languageEnglish
Pages (from-to)307-314
Number of pages8
JournalBritish Journal of Haematology
Volume61
Issue number2
Publication statusPublished - 1985

ASJC Scopus subject areas

  • Hematology

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