Abstract
Thrombotic thrombocytopenic purpura (TTP) is characterized by a reduction in the von Willebrand cleavage protein ADAMTS-13, mainly as a consequence of autoimmunity. Plasma exchange (PEx) is standard, achieving complete remission (CR) in 77-83% of cases, but rates are variable depending on ADAMTS-13 activity and relapse is frequent in patients with <10%. Thus, an effective front-line immunosuppressive treatment is needed. We administered PEx daily until CR and rituximab 100 mg/dose/week for 4 consecutive weeks to 10 patients with a first TTP episode and 1 relapsed patient (8 females (72%) and 3 males (28%)). Median age was 34 years (15-46) and laboratory parameters at diagnosis were as follows: platelets 11 × 10(9)/l (range 7-27.4 × 10(9)/l), lactate dehydrogenase 1822 U/l (range 705-8220 U/l, normal 70-180 U/l), and haemoglobin 6 g/dl (range 4.2-11.8 g/dl). ADAMTS-13 activity was determined in eight patients and was <10% in all. ADAMTS-13 autoantibody titre was determined in seven patients and was >15 units/ml in all (ref: negative <12, undetermined 12-15, positive >15 units/ml); Shiga toxin was negative in all patients. The median number of PEx until CR was 7 (range 4-12); prednisone 1 mg/kg was administered to six patients. T...Continue Reading
References
Aug 8, 1991·The New England Journal of Medicine·G A RockR A Spasoff
Jul 1, 1966·Naika. Internal medicine·Y SugaM Nomura
Jan 20, 2007·British Journal of Haematology·Marie ScullySamuel J Machin
Apr 12, 2008·Haematologica·Francesco ZajaRenato Fanin
Aug 30, 2008·British Journal of Haematology·Drew ProvanRoberto Stasi
Mar 12, 2009·Clinical Infectious Diseases : an Official Publication of the Infectious Diseases Society of America·Melody BenjaminJames N George
Dec 25, 2009·Blood·Johanna A Kremer HovingaJames N George
Feb 24, 2010·Journal of Thrombosis and Haemostasis : JTH·V McDonaldM A Scully
Apr 28, 2010·Annals of Hematology·Jens Marcus ChemnitzChristof Scheid
Jun 16, 2010·European Journal of Haematology·Francesco ZajaRenato Fanin
Aug 6, 2010·Blood·James N George
Aug 24, 2010·Immunological Reviews·Michael J TownsendAndrew C Chan
Sep 16, 2010·Blood·David Gómez-AlmaguerJosé Carlos Jaime-Pérez
Mar 12, 2011·British Journal of Haematology·Daniel HartMarie Scully
Jun 4, 2011·Blood·Marie ScullySamuel J Machin
Sep 20, 2011·Critical Care Medicine·Antoine FroissartUNKNOWN French Thrombotic Microangiopathies Reference Center
Jan 24, 2012·Blood·Wilma BarcelliniAlberto Zanella
Jan 3, 2013·Journal of Thrombosis and Haemostasis : JTH·J-P WestwoodM Scully
Feb 26, 2013·Hematology·Myrna Pequeño-LuévanoDavid Gómez-Almaguer
Mar 9, 2013·European Journal of Haematology·David Gómez-AlmaguerMónica Sánchez-Cárdenas
Sep 17, 2013·European Journal of Haematology·Wilma BarcelliniAlberto Zanella
May 30, 2014·Blood·Miguel HieUNKNOWN French Thrombotic Microangiopathies Reference Centre
Jan 13, 2015·Blood·Wendy LimJames N George
Citations
Oct 16, 2018·Journal of Clinical Apheresis·Emmanuel Bugarin-EstradaDavid Gómez-Almaguer
Nov 10, 2018·Medicine·Rurong SunMin Wu
Aug 5, 2017·Blood·J Evan Sadler
Oct 8, 2019·Intensive Care Medicine·Elie AzoulayUNKNOWN Nine-i Investigators
Feb 12, 2020·Blood Advances·George GoshuaAlfred Ian Lee
Jun 24, 2017·The Journal of International Medical Research·Haifei ChenLongmei Qing
Apr 4, 2020·Expert Review of Hematology·Andrés Gómez-De LeónDavid Gómez-Almaguer
Apr 7, 2017·Nature Reviews. Disease Primers·Johanna A Kremer HovingaKaren Vanhoorelbeke
Feb 4, 2021·Journal of Clinical Apheresis·Amalia Domingo-GonzálezCristina Pascual-Izquierdo
Mar 15, 2021·Transfusion and Apheresis Science : Official Journal of the World Apheresis Association : Official Journal of the European Society for Haemapheresis·César David Galindo-CalvilloDavid Gómez-Almaguer
Apr 26, 2019·Transfusion and Apheresis Science : Official Journal of the World Apheresis Association : Official Journal of the European Society for Haemapheresis·Adrien Picod, Paul Coppo
Nov 9, 2021·Expert Review of Hematology·Katerina PavenskiChristopher J Patriquin