Bleeding signs due to acquired von Willebrand syndrome at diagnosis of chronic myeloid leukaemia in children

Ralf Knöfler, Björn S Lange, Franziska Paul, Oliver Tiebel, Meinolf Suttorp, Ralf Knöfler, Björn S Lange, Franziska Paul, Oliver Tiebel, Meinolf Suttorp

Abstract

A considerable proportion of patients with chronic myeloid leukaemia (CML) may present at diagnosis with high platelet counts. This may result in thrombosis or bleeding complications due to binding of von Willebrand factor (VWF) multimers to platelets. Paediatric CML is very rare and no systematic investigation on clinical complications of elevated platelets has been reported. Data on platelet count and associated haemostaseological complications were retrospectively analysed in a cohort of 156 children with CML. Fifty-one percent (81/156) patients presented with thrombocytosis (platelet count> 500 × 109 /l), and were extreme (>1 000 × 109 /l) in 23/156 (16%). There were no cases of thrombosis but mild bleeding signs were present in 12% (n = 9) children with thrombocytosis. Bleeding occurred without correlation to elevated platelet counts and was associated with reduced large VWF multimers, indicating a diagnosis of acquired von Willebrand syndrome (AVWS), which resolved after initiation of CML treatment. Patients with paediatric CML frequently exhibit high platelet counts not resulting in thrombosis. In patients with thrombocytosis mild bleeding signs due to a low percentage of large VWF multimers can be demonstrated. AVWS may be underdiagnosed in paediatric CML (Clinical-Trials.gov NCT00445822, 9 March 2007).

Keywords: acquired von Willebrand Disease; high platelet count; paediatric CML; thrombocytosis; von Willebrand Multimers.

© 2019 British Society for Haematology and John Wiley & Sons Ltd.

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