PMID: 9432029Jan 31, 1998Paper

Decreased half-life time of plasma von Willebrand factor collagen binding activity in essential thrombocythaemia: normalization after cytoreduction of the increased platelet count

British Journal of Haematology
P J van GenderenJ J Michiels

Abstract

Patients with essential thrombocythaemia (ET) exhibit a decrease of large von Willebrand factor (VWF) multimers in plasma, which is inversely related to the platelet count. In the present study we investigated whether the decrease of large VWF multimers in plasma with increasing platelet counts is the consequence of increased turnover of large VWF multimers in vivo. To that end we measured the half-life times of endogenously released VWF:Ag and VWF:CBA (collagen binding activity) after intravenous administration of desmopressin (DDAVP) to nine ET patients and nine control subjects (N). In addition, the half-life times of VWF:Ag and VWF:CBA were also measured in four ET patients after cytoreduction of the increased platelet count to normal or nearly normal values. Estimated half-life times of VWF:Ag did not differ between ET patients and normals (11.0+/-4.0 h v 12.4+/-2.5 h, P>0.05). Estimated half-life times of VWF:CBA were significantly lower in ET patients as compared with normal individuals (6.1+/-2.0 h v 8.4+/-2.5 h, P<0.05). After cytoreduction of the increased platelet count to (nearly) normal values in all four ET patients the half-life time of VWF:CBA significantly (P=0.014) increased from 5.2+/-1.2 h to 8.7+/-2.0 h. Ou...Continue Reading

Citations

May 24, 2003·Best Practice & Research. Clinical Haematology·Michelle A Elliott, Ayalew Tefferi
Sep 8, 2010·Blood Reviews·Emmanouil PapadakisBenjamin Brenner
Jan 26, 2005·British Journal of Haematology·M A Elliott, A Tefferi
Jun 23, 2010·Journal of Clinical Apheresis·Zbigniew M SzczepiorkowskiUNKNOWN Apheresis Applications Committee of the American Society for Apheresis
Jun 20, 2006·Best Practice & Research. Clinical Haematology·Raffaele LandolfiLinda Novarese
Jun 24, 2008·Expert Opinion on Pharmacotherapy·Naseema Gangat, Ayalew Tefferi
Nov 1, 2001·Best Practice & Research. Clinical Haematology·J J MichielsZ Berneman
Oct 1, 2016·American Journal of Clinical Pathology·Lambert BusqueVikas Gupta
Mar 22, 2000·Clinical and Applied Thrombosis/hemostasis : Official Journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis·J J Michiels
Jul 11, 2006·American Journal of Hematology·Hiroshi Mohri
Nov 30, 2006·American Journal of Hematology·Massimo Franchini, Giuseppe Lippi
Mar 31, 2007·Journal of Clinical Apheresis·Zbigniew M SzczepiorkowskiUNKNOWN Apheresis Applications Committee of the American Society for Apheresis
Mar 17, 2007·Critical Reviews in Clinical Laboratory Sciences·Massimo Franchini, Giuseppe Lippi

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