Proteolysis of von Willebrand factor is decreased in acute promyelocytic leukaemia by treatment with all-trans-retinoic acid

Augusto B. Federici, Anna Falanga, Antonella Lattuada, Nicola Di Rocco, Tiziano Barbui, Pier Mannuccio Mannucci

Research output: Contribution to journalArticlepeer-review

Abstract

Plasma von Willebrand factor (VWF) was investigated in five patients with acute promyelocytic leukaemia (APL) before and after administration of the differentiating agent ail-trans-retinoic acid (ATRA). The purpose of the study was to see how the proteolytic state associated with APL affects VWF structure and function and whether ATRA reverses any abnormality. At the onset of APL, multimeric analysis of plasma VWF revealed a lack of the largest multimers. After ATRA, there was a progressive correction of the multimeric pattern in all cases, with transient appearance of ultralarge multimers in two cases. Proteolysis was investigated with immunopurified and reduced VWF from each patient's plasma. This was electrophoresed and probed with two monoclonal antibodies that identify the 225 kD native subunit and the three native fragments of 189, 176 and 140 kD and differentiate novel proteolytic fragments produced by different proteinases. At the onset of APL, the 225 kD native subunit was relatively decreased, with the appearance of an array of novel VWF proteolytic fragments, ranging in size from

Original languageEnglish
Pages (from-to)733-739
Number of pages7
JournalBritish Journal of Haematology
Volume92
Issue number3
Publication statusPublished - 1996

Keywords

  • Acute promyelocytic leukaemia
  • All-trans-retinoic acid
  • Bleeding complications
  • Proteolysis
  • von Willebrand factor

ASJC Scopus subject areas

  • Hematology

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