More about low-dose rituximab and plasma exchange as front-line therapy for patients with thrombotic thrombocytopenic purpura

Hematology
Alberto Vazquez-MelladoDavid Gomez-Almaguer

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

Jul 1, 1966·Naika. Internal medicine·Y SugaM Nomura
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 24, 2010·Immunological Reviews·Michael J TownsendAndrew C Chan
Feb 26, 2013·Hematology·Myrna Pequeño-LuévanoDavid Gómez-Almaguer
Sep 17, 2013·European Journal of Haematology·Wilma BarcelliniAlberto Zanella
May 30, 2014·Blood·Miguel HieUNKNOWN French Thrombotic Microangiopathies Reference Centre

❮ Previous
Next ❯

Citations

Oct 16, 2018·Journal of Clinical Apheresis·Emmanuel Bugarin-EstradaDavid Gómez-Almaguer
Oct 8, 2019·Intensive Care Medicine·Elie AzoulayUNKNOWN Nine-i Investigators
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
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

❮ Previous
Next ❯

Related Concepts

Related Feeds

Autoimmune Diseases

Autoimmune diseases occur as a result of an attack by the immune system on the body’s own tissues resulting in damage and dysfunction. There are different types of autoimmune diseases, in which there is a complex and unknown interaction between genetics and the environment. Discover the latest research on autoimmune diseases here.

Blood Clotting Disorders

Thrombophilia includes conditions with increased tendency for excessive blood clotting. Blood clotting occurs when the body has insufficient amounts of specialized proteins that make blood clot and stop bleeding. Here is the latest research on blood clotting disorders.