Diagnosis and treatment of immune thrombocytopenia

Czech version

Authors: Libor Červinek
Authors‘ workplace: Interní hematologická a onkologická klinika LF MU a FN Brno, pracoviště Bohunice
Published in: Vnitř Lék 2018; 64(5): 526-529
Category:

Overview

The diagnosis of immune thrombocytopenia consists in the combination of laboratory and clinical pictures of thrombocytopenia while eliminating other disorders characterized by low levels of thrombocytes. The treatment initiation in patients with ITP is recommended when the thrombocyte count has dropped below 20–30 × 10⁹/l, when hemorrhagic manifestations occur and depending on the patient’s risk profile. Corticoids and IVIG are used as first-line treatment. Second-line treatment includes splenectomy, immunosuppressive therapy and administration of thrombopoietin receptor agonists. A new drug in the treatment of ITP is fostamatinib.

Key words:

corticoids – eltrombopag – fostamatinib – immune thrombocytopenia – IVIG – rituximab – romiplostim – sustainable remission – splenectomy

Sources

Rodeghiero F, Stasi R. Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group. Blood 2009; 113(11): 2386–2393. Dostupné z DOI: <http://dx.doi.org/10.1182/blood-2008–07–162503>.
Provan D, Stasi R, Newland A et al. International consensus report on the investigation and management of primary immune thrombocytopenia. Blood 2010; 115(2): 168–186. Dostupné z DOI: <http://dx.doi.org/10.1182/blood-2009–06–225565>.
Neunert C, Lim W, Crowther M et al. The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia. Blood 2011; 117(16): 4190–4207. Dostupné z DOI: <http://dx.doi.org/10.1182/blood-2010–08–302984>.
Portielje J, Westendorp R, Klui-Nelemans H et al. Morbidity and mortality in adults with idiopathic thrombocytopenic purpura. Blood 2001; 97(9): 2549–2554.
Godeau B, Lesage S, Divine M et al. Treatment of adult chronic autoimmune thrombocytopenic purpura with repeated high-dose intravenous immunoglobulin. Blood 1993; 82(5): 1415–1421.
Zoghlami-Rintelen C, Weltrmann A, Bittermann C et al. Efficacy and safety of splenectomy in adult chronic immune thrombocytopenia. Ann Hematol 2003; 82(5): 290–294. Dostupné z DOI: <http://dx.doi.org/10.1007/s00277–002–0550–8>.
Bussel JB, Cheng G, Saleh MN et al. Eltrombopag for the treatment of chronic idiopathic thrombocytopenic purpura. N Engl J Med 2007; 357(22): 2237–2247. Dostupné z DOI: <http://dx.doi.org/10.1056/NEJMoa073275>.
Kuter DJ, Bussel JB, Lyons RM et al. Efficacy of romiplostim in patients with chronic immune thrombocytopenic purpura: a double-blind randomised controlled trial. Lancet 2008; 371(9610): 395–403. Dostupné z DOI: <http://dx.doi.org/10.1016/S0140–6736(08)60203–2>.
Bussel JB, Kuter DJ, Pullarkat V et al. Safety and efficacy of long-term treatment with romiplostim in thrombocytopenic patients with chronic ITP. Blood 2009; 113(10): 2161–2171. Dostupné z DOI: <http://dx.doi.org/10.1182/blood-2008–04–150078>. Erratum in Blood 2009; 113(19): 4822.
Červinek L, Mayer J, Doubek M. Sustained remission of chronic immune thrombocytopenia after discontinuation of treatment with thrombopoietin-receptor agonists in adults. Int J Hematol 2015; 102(1): 7–11. Dostupné z DOI: <http://dx.doi.org/10.1007/s12185–015–1793–1>.
González-López TJ, Pascual C, Álvarez-Román MT et al. Successful discontinuation of eltrombopag after complete remission in patients with primary immune thrombocytopenia. Am J Hematol 2015; 90(3): E40-E43. Dostupné z DOI: <http://dx.doi.org/10.1002/ajh.23900>.
Kappers-Klunne MC, van’t Veer MB. Cyclosporin A for the treatment of patients with chronic idiopathic thrombocytopenic purpura refractory to corticosteroids or splenectomy. Br J Haematol 2001; 114(1): 121–125.
Reiner A, Gernsheimer T, Schlichter SJ. Pulse cyclofosfamide therapy for refractory autoimmune thrombocytopenic purpura. Blood 1995; 85(2): 351–358.
Maloisel F, Andres E, Zimmer J et al. Danazol therapy in patients with chronic idiopathic thrombocytopenic purpura. Am J Med 2004; 116(9): 590–594. Dostupné z DOI: <http://dx.doi.org/10.1016/j.amjmed.2003.12.024>.
Howard J, Hoffbrand AV, Prentice HG et al. Mycophenolate mofetil for the treatment of refractory auto-immune haemolytic anemia and autoimmune thrombocytopenic purpura. Br J Haematol 2002; 117(3): 712–715.
Ghanima W, Khelif A, Waage A et al. Rituximab as second-line treatment for adult immune thrombocytopenia (the RITP trial): a multicentre, randomised, double-blind, placebo-controlled trial. Lancet 2015; 385(9978): 1653–1661. Dostupné z DOI: <http://dx.doi.org/10.1016/S0140–6736(14)61495–1>.