Abstract
Very few data exist on the management of adult patients diagnosed with primary immune thrombocytopenia (ITP). The objectives of this study were to describe the diagnostic and treatment patterns for ITP and to compare the findings to recent ITP guidelines. We retrospectively analyzed the medical records of adult ITP patients diagnosed with primary ITP between January 2011 and June 2012 and examined whether management strategies were consistent or not with eight recent guideline-recommended practices. Overall, median age at the diagnosis of the disease (n = 101) was 58 years and median platelet count 12 × 109/L with 75.2 % of patients having symptoms of ITP. The study perceived two major shortcomings in the diagnostic approach: (1) failure to perform peripheral blood film examination in 22.8 % of patients, a test that is mandatory by all guidelines, and (2) ordinary bone marrow assessment in more than half of the patients at diagnosis (50.5 %), a test not routinely recommended by guidelines. Low appropriateness in therapeutic management of patients included (1) unjustified use of intravenous immunoglobulin in the absence of bleeding in 54.8 % of patients and (2) splenectomy not being deferred until 6–12 months from diagnosis (median 161 days). Data also reflect a trend towards the early use of thrombopoietin receptor agonists in the treatment of patients who are refractory to any first-line therapy. We have recognized important areas of inapropriateness in the diagnostic and therapeutic management of adult ITP patients. Compliance with established guidelines should be encouraged in order to improve patient outcomes.
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Rodeghiero F, Stasi R, Gernsheimer T, Michel M, Provan D, Arnold DM, Bussel JB, Cines DB, Chong BH, Cooper N, Godeau B, Lechner K, Mazzucconi MG, McMillan R, Sanz MA, Imbach P, Blanchette V, Kühne T, Ruggeri M, George JN (2009) Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group. Blood 113:2386–2393
Provan D, Stasi R, Newland AC, Blanchette VS, Bolton-Maggs P, Bussel JB, Chong BH, Cines DB, Gernsheimer TB, Godeau B, Grainger J, Greer I, Hunt BJ, Imbach PA, Lyons G, McMillan R, Rodeghiero F, Sanz MA, Tarantino M, Watson S, Young J, Kuter DJ (2010) International consensus report on the investigation and management of primary immune thrombocytopenia. Blood 115:168–186
Matzdorff A, Giagounidis A, Greinacher A, Hiller E, Kiefel V, Müller-Beissenhirtz H, Ostermann H, Rummel M, Sachs UJ, Salama A (2010) Diagnosis and therapy of autoimmune thrombocytopenia. Recommendations of a joint Expert Group of DGHO, DGTI, DTH. Onkologie 33(Suppl 3):2–20
Ghanima W, Holme PA, Tjønnfjord GE (2010) Immune thrombocytopenia—pathophysiology and treatment. Tidsskr Nor Laegeforen 130:2120–2123
Neunert C, Lim W, Crowther M, Cohen A, Solberg L Jr, Crowther MA (2011) The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia. Blood 117:4190–4207
Sanz MÁ, Vicente García V, Fernández A, López MF, Grande C, Jarque I, Martínez R, Mingot ME, Monteagudo E, Ribera JM, Valcárcel D; Sociedad Española de Hematología y Hemoterapia; Sociedad Española de Hematología y Oncología Pediátricas (2012) [Guidelines for diagnosis, treatment and monitoring of primary immune thrombocytopenia]. Med Clin (Barc) 138:261.e1-261.e17
Pabinger I, Gastl G, Steurer M, Sormann S, Fillitz M, Friedl J, Geissler D, Geissler K, Greil R, Knöbl P, Kozek-Langenecker S, Krippl P, Kyrle P, Lang A, Linkesch W, Ludwig H, Müller M, Panzer S, Pittermann E, Thaler J, Weltermann A (2012) Primary immune thrombocytopenia in adults: diagnostics and treatment consensus statement of the Austrian Society of Hematology and Oncology (ÖGHO). Wien Klin Wochenschr 124:111–123
Nationella ITP-gruppe. Wadenvik H, coord. Primär immunologisk trombocytopeni (ITP) hos vuxna. Nationella rekommendationer för utredning och behand-ling. Uppdateras senast 2011. Estocolmo: Nationella Riktlinjer. Available at http://www.sfhem.se/Filarkiv
Collège de la Haute Autorité de Santé. Purpura thrombopénique immunologique de l’enfant et de l’adulte. Saint-Denis La-Plaine Cedex: Haute Autorité de Santé; 2009. http://www.has-sante.fr/portail/upload/docs/application/pdf/2009-12/ald_2_pnds_pti_imune_enft_adulte_web.pdf
Grande García C, Martínez Martínez R, Valcarcel Ferreiras D, Grupo de estudio de Trombocitopenia Inmune Primaria de la Sociedad Española de Hematología y Hemoterapia y Sociedad Española de Trombosis y Hemostasia (2014) Comparison of international guidelines for primary autoimmune thrombocytopenia. Med Clin (Barc) 143:408–419
Lee LH, Caguioa P, Chin NS, Chiou TJ, Lee JW, Miyakawa Y, Tambunan KL, Chong BH (2011) Chronic adult primary immune thrombocytopenia (ITP) in the Asia-Pacific region. Int J Hematol 94:142–149
Lu KH, George JN, Vesely SK, Terrell DR (2014) Management of primary immune thrombocytopenia, 2012: a survey of Oklahoma hematologists-oncologists. Am J Med Sci 347:190–194
Meillón-García LA, García-Chávez J, Gómez-Almaguer D, Gutiérrez-Espíndola GR, Martínez-Murillo C (2014) Primary immune thrombocytopenia in adults in Mexico: national characteristics and the relation to international literature. Gac Med Mex 150:279–288
Schultz CL, Mitra N, Schapira MM, Lambert MP (2014) Influence of the American Society of Hematology guidelines on the management of newly diagnosed childhood immune thrombocytopenia. JAMA Pediatr 168, e142214
Terrell DR, Beebe LA, Vesely SK, Neas BR, Segal JB, George JN (2010) The incidence of immune thrombocytopenic purpura in children and adults: a critical review of published reports. Am J Hematol 85:174–180
Rodeghiero F, Ruggeri M (2014) ITP and international guidelines: what do we know, what do we need? Presse Med 43:e61–7
Page LK, Psaila B, Provan D, Michael Hamilton J, Jenkins JM, Elish AS, Lesser ML, Bussel JB (2007) The immune thrombocytopenic purpura (ITP) bleeding score: assessment of bleeding in patients with ITP. Br J Haematol 138:245–248
Neunert C, Noroozi N, Norman G, Buchanan GR, Goy J, Nazi I, Kelton JG, Arnold DM (2015) Severe bleeding events in adults and children with primary immune thrombocytopenia: a systematic review. J Thromb Haemost 13:457–464
Fogarty PF (2009) Chronic immune thrombocytopenia in adults: epidemiology and clinical presentation. Hematol Oncol Clin North Am 23:1213–1221
Neylon AJ, Saunders PW, Howard MR, Proctor SJ, Taylor PR, Northern Region Haematology Group (2003) Clinically significant newly presenting autoimmune thrombocytopenic purpura in adults: a prospective study of a population-based cohort of 245 patients. Br J Haematol 122:966–974
Michel M (2013) Immune thrombocytopenia nomenclature, consensus reports, and guidelines: what are the consequences for daily practice and clinical research? Semin Hematol 50(Suppl 1):S50–54
Rodeghiero F, Besalduch J, Michel M, Provan D, Grotzinger K, Thompson G (2010) Treatment practices in adults with chronic immune thrombocytopenia—a European perspective. Eur J Haematol 84:160–168
Wei Y, Ji XB, Wang YW, Wang JX, Yang EQ, Wang ZC, Sang YQ, Bi ZM, Ren CA, Zhou F, Liu GQ, Peng J, Hou M (2016) High-dose dexamethasone vs prednisone for treatment of adult immune thrombocytopenia: a prospective multicenter randomized trial. Blood 127:296–302
Rodeghiero F, Ruggeri M (2008) Is splenectomy still the gold standard for the treatment of chronic ITP? Am J Hematol 83:91
Ghanima W, Godeau B, Cines DB, Bussel JB (2012) How I treat immune thrombocytopenia: the choice between splenectomy or a medical therapy as a second-line treatment. Blood 120:960–969
Stasi R, Stipa E, Masi M, Cecconi M, Scimò MT, Oliva F, Sciarra A, Perrotti AP, Adomo G, Amadori S, Papa G (1995) Long-term observation of 208 adults with chronic idiopathic thrombocytopenic purpura. Am J Med 98:436–442
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This work was supported by Amgen S.A. Spain. Amgen did not have any role in study design, collection, analysis, and interpretation of data, writing the report, or in the decision to submit the report for publication. The authors thank Juan Luis Sanz (APICES, Madrid, Spain) for his contribution in the data management and statistical analysis of this study and for critically reviewing the manuscript.
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Lozano, M.L., Revilla, N., Gonzalez-Lopez, T.J. et al. Real-life management of primary immune thrombocytopenia (ITP) in adult patients and adherence to practice guidelines. Ann Hematol 95, 1089–1098 (2016). https://doi.org/10.1007/s00277-016-2665-3
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DOI: https://doi.org/10.1007/s00277-016-2665-3