Update on diagnosis and treatment of immune thrombocytopenia

References

• Yuan YP, Yang X, Chen YJ. Research Advances on the Pathogenesis of Primary Immune Thrombocytopenia–Review. Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2019;27(5):1706–1710.
   PubMedGoogle Scholar
• Cines DB, Liebman H, Stasi R. Pathobiology of secondary immune thrombocytopenia. Semin Hematol. 2009;46(1 Suppl 2):S2–14.
   PubMed Web of Science ®Google Scholar
• Liebman HA. Recognizing and treating secondary immune thrombocytopenic purpura associated with lymphoproliferative disorders. Semin Hematol. 2009;46(1 Suppl 2):S33–6.
   PubMed Web of Science ®Google Scholar
• Kado R, McCune WJ. Treatment of primary and secondary immune thrombocytopenia. Curr Opin Rheumatol. 2019;31(3):213–222.
   PubMed Web of Science ®Google Scholar
• Sultan S, Ahmed SI, Murad S, et al. Primary versus secondary immune thrombocytopenia in adults; a comparative analysis of clinical and laboratory attributes in newly diagnosed patients in Southern Pakistan. Med J Malaysia. 2016;71(5):269–274.
   PubMedGoogle Scholar
• Rodeghiero F, Stasi R, Gernsheimer T, et al. 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. .
   PubMed Web of Science ®Google Scholar
• Neunert C, Terrell DR, Arnold DM, et al. American Society of Hematology 2019 guidelines for immune thrombocytopenia. Blood Adv. 2019;3(23):3829–3866. .
   PubMed Web of Science ®Google Scholar
• Provan D, Arnold DM, Bussel JB, et al. Updated international consensus report on the investigation and management of primary immune thrombocytopenia. Blood Adv. 2019;3(22):3780–3817.
   PubMed Web of Science ®Google Scholar
• Audia S, Mahévas M, Samson M, et al. Pathogenesis of immune thrombocytopenia. Autoimmun Rev. 2017;16(6):620–632.
   PubMed Web of Science ®Google Scholar
• Rodeghiero F, Michel M, Gernsheimer T, et al. Standardization of bleeding assessment in immune thrombocytopenia: report from the International Working Group. Blood. 2013;121(14):2596–2606. .
   PubMed Web of Science ®Google Scholar
• Fogarty PF, Tarantino MD, Brainsky A, et al. Selective validation of the WHO Bleeding Scale in patients with chronic immune thrombocytopenia. Curr Med Res Opin. 2012;28(1):79–87.
   PubMed Web of Science ®Google Scholar
• Provan D, Stasi R, Newland AC, et al. International consensus report on the investigation and management of primary immune thrombocytopenia. Blood. 2010;115(2):168–186. .
   PubMed Web of Science ®Google Scholar
• Bastida JM, Benito R, Lozano ML, et al. Molecular Diagnosis of Inherited Coagulation and Bleeding Disorders. Semin Thromb Hemost. 2019;45(7):695–707.
   PubMed Web of Science ®Google Scholar
• Mishra K, Jandial A, Malhotra P, et al. Wet purpura: a sinister sign in thrombocytopenia. BMJ Case Rep. 2017 Sep 1;2017.
   Google Scholar
• Ren DQ, Gao H, Li ZC. [Prognostic significance of peripheral blood cell and bone marrow megakaryocyte counts in patients with idiopathic thrombocytopenic purpura]. Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2003;11(2):199–201.
   PubMedGoogle Scholar
• Tseng V, Morgan AS, Leith CP, et al. Efficient assessment of peripheral blood lymphocytosis in adults: developing new thresholds for blood smear review by pathologists. Clin Chem Lab Med. 2014;52(12):1763–1770.
   PubMed Web of Science ®Google Scholar
• Wongrakpanich S, Salahuddin M, Mittar P. An abnormal peripheral blood smear and altered mental status. Cleve Clin J Med. 2016;83(9):643–644.
   PubMed Web of Science ®Google Scholar
• Balduini CL, Pecci A, Savoia A. Recent advances in the understanding and management of MYH9-related inherited thrombocytopenias. Br J Haematol. 2011 Jul;154(2):161-174.
   Google Scholar
• Hamad H, Sahu KK, Dunn S, et al. Rifampin Induced Thrombotic Thrombocytopenic Purpura. Indian J Hematol Blood Transfus. 2020;36(3):575–577.
   PubMed Web of Science ®Google Scholar
• Altintas A, Ozel A, Okur N, et al. Prevalence and clinical significance of elevated antinuclear antibody test in children and adult patients with idiopathic thrombocytopenic purpura. J Thromb Thrombolysis. 2007;24(2):163–168. .
   PubMed Web of Science ®Google Scholar
• Moulis G, Comont T, Germain J, et al. Significance of antinuclear antibodies in primary immune thrombocytopenia: results of the CARMEN registry. Blood Adv. 2020;4(9):1974–1977. .
   PubMed Web of Science ®Google Scholar
• Neunert CE, Cooper N. Evidence-based management of immune thrombocytopenia: ASH guideline update. Hematol Am Soc Hematol Educ Program. 2018;2018(1):568–575.
   PubMed Web of Science ®Google Scholar
• Heng LL, Caguioa P, Chin NS, et al. Chronic adult primary immune thrombocytopenia (ITP) in the Asia-Pacific region. Int J Hematol. 2011;94(2):142–149.
   PubMed Web of Science ®Google Scholar
• Sheema K, Ikramdin U, Arshi N, et al. Role of Helicobacter pylori Eradication Therapy on Platelet Recovery in Chronic Immune Thrombocytopenic Purpura. Gastroenterol Res Pract. 2017;2017:9529752.
   PubMed Web of Science ®Google Scholar
• Frydman GH, Davis N, Beck PL, et al. Helicobacter pylori Eradication in Patients with Immune Thrombocytopenic Purpura: a Review and the Role of Biogeography. Helicobacter. 2015;20(4):239–251.
   PubMed Web of Science ®Google Scholar
• Varma S, Kumar S, Garg A, et al. Hepatitis C virus infection among patients with chronic immune thrombocytopenic purpura in northern India. J Clin Exp Hepatol. 2011;1(2):68–72. .
   PubMedGoogle Scholar
• Franchini M, Veneri D, Lippi G. Thrombocytopenia and infections. Expert Rev Hematol. 2017;10(1):99–106.
   PubMed Web of Science ®Google Scholar
• Joo EJ, Chang Y, Yeom JS, et al. B infection is associated with an increased incidence of thrombocytopenia in healthy adults without cirrhosis. J Viral Hepat. 2017;24(3):253–258.
   PubMed Web of Science ®Google Scholar
• Tang YT, He P, Li YZ, et al. Diagnostic value of platelet indices and bone marrow megakaryocytic parameters in immune thrombocytopenic purpura. Blood Coagul Fibrin. 2017;28(1):83–90. .
   PubMed Web of Science ®Google Scholar
• Ahmad Z, Durrani NU, Hazir T. Bone marrow examination in ITP in children: is it mandatory? J Coll Physicians Surg Pak. 2007;17(6):347–349.
   PubMed Web of Science ®Google Scholar
• Salama A, Kiefel V, Amberg R, et al. Treatment of autoimmune thrombocytopenic purpura with rhesus antibodies (anti-Rh0(D). Blut. 1984;49(1):29–35.
   PubMedGoogle Scholar
• McMillan R. Antiplatelet antibodies in chronic adult immune thrombocytopenic purpura: assays and epitopes. J Pediatr Hematol Oncol. 2003;25(Suppl 1):S57–61.
   PubMed Web of Science ®Google Scholar
• McMillan R. Antiplatelet antibodies in chronic immune thrombocytopenia and their role in platelet destruction and defective platelet production. Hematol Oncol Clin North Am. 2009;23(6):1163–1175.
   PubMed Web of Science ®Google Scholar
• Chan H, Moore JC, Finch CN, et al. The IgG subclasses of platelet-associated autoantibodies directed against platelet glycoproteins IIb/IIIa in patients with idiopathic thrombocytopenic purpura. Br J Haematol. 2003;122(5):818–824.
   PubMed Web of Science ®Google Scholar
• Sahu KK, Jindal V, Anderson J, et al. Current Perspectives on Diagnostic Assays and Anti-PF4 Antibodies for the Diagnosis of Heparin-Induced Thrombocytopenia. J Blood Med. 2020;11:267–277.
   PubMed Web of Science ®Google Scholar
• Stasi R, Stipa E, Masi M, et al. Prevalence and clinical significance of elevated antiphospholipid antibodies in patients with idiopathic thrombocytopenic purpura. Blood. 1994;84(12):4203–4208. .
   PubMed Web of Science ®Google Scholar
• Pierrot-Deseilligny Despujol C, Michel M, Khellaf M, et al. Antiphospholipid antibodies in adults with immune thrombocytopenic purpura. Br J Haematol. 2008;142(4):638–643. .
   PubMed Web of Science ®Google Scholar
• Yang YJ, Yun GW, Song IC, et al. Clinical implications of elevated antiphospholipid antibodies in adult patients with primary immune thrombocytopenia. Korean J Intern Med. 2011;26(4):449–454. .
   PubMedGoogle Scholar
• Marta GN, De Campos FP. Immune thrombocytopenia and autoimmune thyroid disease: a controversial overlap. Autops Case Rep. 2015;5(2):45–48.
   PubMedGoogle Scholar
• Ioachimescu AG, Makdissi A, Lichtin A, et al. Thyroid disease in patients with idiopathic thrombocytopenia: a cohort study. Thyroid. 2007;17(11):1137–1142.
   PubMed Web of Science ®Google Scholar
• Cheung E, Liebman HA. Thyroid disease in patients with immune thrombocytopenia. Hematol Oncol Clin North Am. 2009;23(6):1251–1260.
   PubMed Web of Science ®Google Scholar
• Giordano P, Urbano F, Lassandro G, et al. Role of antithyroid autoimmunity as a predictive biomarker of chronic immune thrombocytopenia. Pediatr Blood Cancer. 2019;66(1):e27452. .
   PubMed Web of Science ®Google Scholar
• Abe Y, Wada H, Tomatsu H, et al. A simple technique to determine thrombopoiesis level using immature platelet fraction (IPF). Thromb Res. 2006;118(4):463–469. .
   PubMed Web of Science ®Google Scholar
• Mishra K, Jandial A, Sandal R, et al. Poor Platelet Function on Sonoclot Signature Is Associated with High Incidence of Bleeding in Severe Immune Thrombocytopenia. Blood. 2018;132(Supplement 1):4991. .
   Google Scholar
• Mishra K, Malhotra P, Jandial A, et al. Bleeding Risk Assessment By Sonoclot in Severe Immune Thrombocytopenia. Blood. 2017;13(Supplement 1):2320. hio0
   Google Scholar
• Mizutani H, Furubayashi T, Imai Y, et al. Mechanisms of corticosteroid action in immune thrombocytopenic purpura (ITP): experimental studies using ITP-prone mice, (NZW x BXSB) F1. Blood. 1992;79(4):942–947. .
   PubMed Web of Science ®Google Scholar
• Cheng Y, Wong RS, Soo YO, et al. Initial treatment of immune thrombocytopenic purpura with high-dose dexamethasone. N Engl J Med. 2003;349(9):831–836. .
   PubMed Web of Science ®Google Scholar
• Borst F, Keuning JJ, Van Hulsteijn H, et al. High-dose dexamethasone as a first- and second-line treatment of idiopathic thrombocytopenic purpura in adults. Ann Hematol. 2004;83(12):764–768.
   PubMed Web of Science ®Google Scholar
• Mithoowani S, Gregory-Miller K, Goy J, et al. High-dose dexamethasone compared with prednisone for previously untreated primary immune thrombocytopenia: a systematic review and meta-analysis. Lancet Haematol. 2016;3(10):e489–e96. .
   PubMed Web of Science ®Google Scholar
• Godeau B, Chevret S, Varet B, et al. Intravenous immunoglobulin or high-dose methylprednisolone, with or without oral prednisone, for adults with untreated severe autoimmune thrombocytopenic purpura: a randomised, multicentre trial. Lancet. 2002;359(9300):23–29. . London, England
   PubMed Web of Science ®Google Scholar
• Godeau B, Zini JM, Schaeffer A, et al. High-dose methylprednisolone is an alternative treatment for adults with autoimmune thrombocytopenic purpura refractory to intravenous immunoglobulins and oral corticosteroids. Am J Hematol. 1995;48(4):282–284.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMed Web of Science ®Google Scholar
• Neylon AJ, Saunders PW, Howard MR, et al. Clinically significant newly presenting autoimmune thrombocytopenic purpura in adults: a prospective study of a population-based cohort of 245 patients. Br J Haematol. 2003;122(6):966–974.
   PubMed Web of Science ®Google Scholar
• Stasi R, Stipa E, Masi M, et al. Long-term observation of 208 adults with chronic idiopathic thrombocytopenic purpura. Am J Med. 1995;98(5):436–442. .
   PubMed Web of Science ®Google Scholar
• Guidry JA, George JN, Vesely SK, et al. Corticosteroid side-effects and risk for bleeding in immune thrombocytopenic purpura: patient and hematologist perspectives. Eur J Haematol. 2009;83(3):175–182.
   PubMed Web of Science ®Google Scholar
• Guidry JA, Watson S, George JN, et al. Addendum to corticosteroid side effects and risk for bleeding in immune thrombocytopenic purpura: patient perspectives. Eur J Haematol. 2009;83(5):497–498.
   PubMed Web of Science ®Google Scholar
• Buckley L, Guyatt G, Fink HA, et al. American College of Rheumatology Guideline for the Prevention and Treatment of Glucocorticoid-Induced Osteoporosis. Arthritis Rheumatol. 2017;69(8):1521–1537. . Hoboken, NJ. 2017
   PubMed Web of Science ®Google Scholar
• Messer J, Reitman D, Sacks HS, et al. Association of adrenocorticosteroid therapy and peptic-ulcer disease. N Engl J Med. 1983;309(1):21–24.
   PubMed Web of Science ®Google Scholar
• Imbach P, Barandun S, d’Apuzzo V, et al. High-dose intravenous gammaglobulin for idiopathic thrombocytopenic purpura in childhood. Lancet. 1981;1(8232):1228–1231. . London, England
   PubMed Web of Science ®Google Scholar
• Lazarus AH, Crow AR. Mechanism of action of IVIG and anti-D in ITP. Transfus Apher Sci. 2003;28(3):249–255.
   PubMed Web of Science ®Google Scholar
• Lazarus AH. Mechanism of action of IVIG in ITP. Vox Sang. 2002;83(Suppl 1):53–55.
   PubMedGoogle Scholar
• Salib M, Clayden R, Clare R, et al. Difficulties in establishing the diagnosis of immune thrombocytopenia: an agreement study. Am J Hematol. 2016;91(8):E327–9. .
   PubMed Web of Science ®Google Scholar
• Hong J, Bang SM, Mun YC, et al. Efficacy and Safety of a New 10% Intravenous Immunoglobulin Product in Patients with Primary Immune Thrombocytopenia (ITP). J Korean Med Sci. 2018;33(19):e142. .
   PubMed Web of Science ®Google Scholar
• Ammann EM, Haskins CB, Fillman KM, et al. Intravenous immune globulin and thromboembolic adverse events: a systematic review and meta-analysis of RCTs. Am J Hematol. 2016;91(6):594–605. .
   PubMed Web of Science ®Google Scholar
• Ware RE, Zimmerman SA. Anti-D: mechanisms of action. Semin Hematol. 1998;35(1 Suppl 1):14–22.
   PubMed Web of Science ®Google Scholar
• Cooper N. Intravenous immunoglobulin and anti-RhD therapy in the management of immune thrombocytopenia. Hematol Oncol Clin North Am. 2009;23(6):1317–1327.
   PubMed Web of Science ®Google Scholar
• Scaradavou A, Woo B, Woloski BM, et al. Intravenous anti-D treatment of immune thrombocytopenic purpura: experience in 272 patients. Blood. 1997;89(8):2689–2700. .
   PubMed Web of Science ®Google Scholar
• Naithani R, Kumar R, Mahapatra M, et al. Efficacy and safety of anti-D for treatment of adults with immune thrombocytopenia. Platelets. 2009;20(7):525–527.
   PubMed Web of Science ®Google Scholar
• Freiberg A, Mauger D. Efficacy, safety, and dose response of intravenous anti-D immune globulin (WinRho SDF) for the treatment of idiopathic thrombocytopenic purpura in children. Semin Hematol. 1998;35(1 Suppl 1):23–27.
   PubMed Web of Science ®Google Scholar
• Gaines AR. Acute onset hemoglobinemia and/or hemoglobinuria and sequelae following Rh(o)(D) immune globulin intravenous administration in immune thrombocytopenic purpura patients. Blood. 2000;95(8):2523–2529.
   PubMed Web of Science ®Google Scholar
• Hong F, Ruiz R, Price H, et al. Safety profile of WinRho anti-D. Semin Hematol. 1998;35(1 Suppl 1):9–13.
   PubMed Web of Science ®Google Scholar
• Khellaf M, Viallard JF, Hamidou M, et al. A retrospective pilot evaluation of switching thrombopoietic receptor-agonists in immune thrombocytopenia. Haematologica. 2013;98(6):881–887. .
   PubMed Web of Science ®Google Scholar
• Cragg MS, Walshe CA, Ivanov AO, et al. The biology of CD20 and its potential as a target for mAb therapy. Curr Directions Autoimmunity. 2005;8:140–174.
   PubMedGoogle Scholar
• Zaja F, Vianelli N, Volpetti S, et al. Low-dose rituximab in adult patients with primary immune thrombocytopenia. Eur J Haematol. 2010;85(4):329–334. .
   PubMed Web of Science ®Google Scholar
• Mishra K, Kumar S, Jandial A, et al. Real-world Experience of Rituximab in Immune Thrombocytopenia. Indian J Hematol Blood Transfus. 2020 (accepted). https://doi.org/10.1007/s12288-020-01351–3
   Google Scholar
• Cheng G, Saleh MN, Marcher C, et al. Eltrombopag for management of chronic immune thrombocytopenia (RAISE): a 6-month, randomised, phase 3 study. Lancet. 2011;377(9763):393–402. . London, England
   PubMed Web of Science ®Google Scholar
• Tran H, Brighton T, Grigg A, et al. A multi-centre, single-arm, open-label study evaluating the safety and efficacy of fixed dose rituximab in patients with refractory, relapsed or chronic idiopathic thrombocytopenic purpura (R-ITP1000 study). Br J Haematol. 2014;167(2):243–251. .
   PubMed Web of Science ®Google Scholar
• Miyakawa Y, Katsutani S, Yano T, et al. Efficacy and safety of rituximab in Japanese patients with relapsed chronic immune thrombocytopenia refractory to conventional therapy. Int J Hematol. 2015;102(6):654–661. .
   PubMed Web of Science ®Google Scholar
• Marangon M, Vianelli N, Palandri F, et al. Rituximab in immune thrombocytopenia: gender, age, and response as predictors of long-term response. Eur J Haematol. 2017;98(4):371–377. .
   PubMed Web of Science ®Google Scholar
• 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. . London, England
   PubMed Web of Science ®Google Scholar
• Sys J, Provan D, Schauwvlieghe A, et al. The role of splenectomy in autoimmune hematological disorders: outdated or still worth considering? Blood Rev. 2017;31(3):159–172.
   PubMed Web of Science ®Google Scholar
• Pasa S, Altintas A, Cil T, et al. The efficacy of rituximab in patients with splenectomized refractory chronic idiopathic thrombocythopenic purpura. J Thromb Thrombolysis. 2009;27(3):329–333.
   PubMed Web of Science ®Google Scholar
• Hindilerden F, Yönal-Hindilerden İ, Yenerel MN, et al. Rituximab Therapy in Adults with Refractory Symptomatic Immune Thrombocytopenia: long-Term Follow-Up of 15 Cases. Turk J Haematol. 2017;34(1):72–80.
   PubMed Web of Science ®Google Scholar
• Qu M, Zhou J, Yang SJ, et al. Efficacy and safety of rituximab for minors with immune thrombocytopenia: a systematic review and meta-analysis. J Int Med Res. 2020;48(10):300060520962348.
   PubMed Web of Science ®Google Scholar
• Deshayes S, Khellaf M, Zarour A, et al. Long-term safety and efficacy of rituximab in 248 adults with immune thrombocytopenia: results at 5 years from the French prospective registry ITP-ritux. Am J Hematol. 2019;94(12):1314–1324. .
   PubMed Web of Science ®Google Scholar
• Paul F, Cartron G. Infusion-related reactions to rituximab: frequency, mechanisms and predictors. Expert Rev Clin Immunol. 2019;15(4):383–389.
   PubMed Web of Science ®Google Scholar
• Sahu KK, Petrou N, Cohn Z, et al. Rituximab-induced late-onset neutropenia. BMJ Case Rep. 2019;12(12):12.
   Google Scholar
• Focosi D, Tuccori M, Maggi F. Progressive multifocal leukoencephalopathy and anti-CD20 monoclonal antibodies: what do we know after 20 years of rituximab. Rev Med Virol. 2019;29(6):e2077.
   PubMed Web of Science ®Google Scholar
• Tomiyama Y, Miyakawa Y, Okamoto S, et al. A lower starting dose of eltrombopag is efficacious in Japanese patients with previously treated chronic immune thrombocytopenia. J Thromb Haemost. 2012;10(5):799–806. .
   PubMed Web of Science ®Google Scholar
• Mishra K, Pramanik S, Jandial A, et al. Real-world experience of eltrombopag in immune thrombocytopenia. Am J Blood Res. 2020;10(5):240–251.
   PubMed Web of Science ®Google Scholar
• Saleh MN, Bussel JB, Cheng G, et al. Safety and efficacy of eltrombopag for treatment of chronic immune thrombocytopenia: results of the long-term, open-label EXTEND study. Blood. 2013;121(3):537–545. .
   PubMed Web of Science ®Google Scholar
• Bussel JB, Provan D, Shamsi T, et al. Effect of eltrombopag on platelet counts and bleeding during treatment of chronic idiopathic thrombocytopenic purpura: a randomised, double-blind, placebo-controlled trial. Lancet. 2009;373(9664):641–648. . London, England
   PubMed Web of Science ®Google Scholar
• Cooper N, Ghanima W, Solomon CG. Immune Thrombocytopenia. N Engl J Med. 2019;381(10):945–955.
   PubMed Web of Science ®Google Scholar
• Ghanima W, Geyer JT, Lee CS, et al. Bone marrow fibrosis in 66 patients with immune thrombocytopenia treated with thrombopoietin-receptor agonists: a single-center, long-term follow-up. Haematologica. 2014;99(5):937–944. .
   PubMed Web of Science ®Google Scholar
• Sperati CJ, Streiff MB. Acute renal failure in a patient with antiphospholipid syndrome and immune thrombocytopenic purpura treated with eltrombopag. Am J Hematol. 2010;85(9):724–726.
   PubMed Web of Science ®Google Scholar
• Cheng G. Thrombopoietin Receptor Agonists for the treatment of ITP. Japan J Clin Hematol. 2013;54(10):1915–1918. Rinsho ketsueki
   PubMedGoogle Scholar
• Newland A, Caulier MT, Kappers-Klunne M, et al. An open-label, unit dose-finding study of AMG 531, a novel thrombopoiesis-stimulating peptibody, in patients with immune thrombocytopenic purpura. Br J Haematol. 2006;135(4):547–553. .
   PubMed Web of Science ®Google Scholar
• Molineux G, Newland A. Development of romiplostim for the treatment of patients with chronic immune thrombocytopenia: from bench to bedside. Br J Haematol. 2010;150(1):9–20.
   PubMed Web of Science ®Google Scholar
• Broudy VC, Lin NL. AMG531 stimulates megakaryopoiesis in vitro by binding to Mpl. Cytokine. 2004;25(2):52–60.
   PubMed Web of Science ®Google Scholar
• Kuter DJ, Rummel M, Boccia R, et al. Romiplostim or standard of care in patients with immune thrombocytopenia. N Engl J Med. 2010;363(20):1889–1899. .
   PubMed Web of Science ®Google Scholar
• Cines DB, Wasser J, Rodeghiero F, et al. Safety and efficacy of romiplostim in splenectomized and nonsplenectomized patients with primary immune thrombocytopenia. Haematologica. 2017;102(8):1342–1351. .
   PubMed Web of Science ®Google Scholar
• Shirasugi Y, Ando K, Miyazaki K, et al. Romiplostim for the treatment of chronic immune thrombocytopenia in adult Japanese patients: a double-blind, randomized Phase III clinical trial. Int J Hematol. 2011;94(1):71–80. .
   PubMed Web of Science ®Google Scholar
• Bidika E, Fayyaz H, Salib M, et al. Romiplostim and Eltrombopag in Immune Thrombocytopenia as a Second-Line Treatment. Cureus. 2020;12(8):e9920.
   PubMed Web of Science ®Google Scholar
• Puavilai T, Thadanipon K, Rattanasiri S, et al. Treatment efficacy for adult persistent immune thrombocytopenia: a systematic review and network meta-analysis. Br J Haematol. 2020;188(3):450–459. .
   PubMed Web of Science ®Google Scholar
• Kuter DJ, Bussel JB, Newland A, et al. Long-term treatment with romiplostim in patients with chronic immune thrombocytopenia: safety and efficacy. Br J Haematol. 2013;161(3):411–423. .
   PubMed Web of Science ®Google Scholar
• Janssens A, Rodeghiero F, Anderson D, et al. Changes in bone marrow morphology in adults receiving romiplostim for the treatment of thrombocytopenia associated with primary immune thrombocytopenia. Ann Hematol. 2016;95(7):1077–1087. .
   PubMed Web of Science ®Google Scholar
• Jurczak W, Chojnowski K, Mayer J, et al. Phase 3 randomised study of avatrombopag, a novel thrombopoietin receptor agonist for the treatment of chronic immune thrombocytopenia. Br J Haematol. 2018;183(3):479–490. .
   PubMed Web of Science ®Google Scholar
• Długosz-Danecka M, Zdziarska J, Jurczak W. Avatrombopag for the treatment of immune thrombocytopenia. Expert Rev Clin Immunol. 2019;15(4):327–339.
   PubMed Web of Science ®Google Scholar
• Bussel JB, Kuter DJ, Aledort LM, et al. A randomized trial of avatrombopag, an investigational thrombopoietin-receptor agonist, in persistent and chronic immune thrombocytopenia. Blood. 2014;123(25):3887–3894. .
   PubMed Web of Science ®Google Scholar
• Xu H, Cai R. Avatrombopag for the treatment of thrombocytopenia in patients with chronic liver disease. Expert Rev Clin Pharmacol. 2019;12(9):859–865.
   PubMed Web of Science ®Google Scholar
• González-Porras JR, Mingot-Castellano ME, Andrade MM, et al. Use of eltrombopag after romiplostim in primary immune thrombocytopenia. Br J Haematol. 2015;169(1):111–116. .
   PubMed Web of Science ®Google Scholar
• Chater C, Terriou L, Duhamel A, et al. Reemergence of Splenectomy for ITP Second-line Treatment? Ann Surg. 2016;264(5):772–777. .
   PubMed Web of Science ®Google Scholar
• Rodeghiero F. A critical appraisal of the evidence for the role of splenectomy in adults and children with ITP. Br J Haematol. 2018;181(2):183–195.
   PubMed Web of Science ®Google Scholar
• Wu JM, Lai IR, Yuan RH, et al. Laparoscopic splenectomy for idiopathic thrombocytopenic purpura. Am J Surg. 2004;187(6):720–723.
   PubMed Web of Science ®Google Scholar
• George JN, Woolf SH, Raskob GE, et al. Idiopathic thrombocytopenic purpura: a practice guideline developed by explicit methods for the American Society of Hematology. Blood. 1996;88(1):3–40. .
   PubMed Web of Science ®Google Scholar
• Kojouri K, Vesely SK, Terrell DR, et al. Splenectomy for adult patients with idiopathic thrombocytopenic purpura: a systematic review to assess long-term platelet count responses, prediction of response, and surgical complications. Blood. 2004;104(9):2623–2634.
   PubMed Web of Science ®Google Scholar
• Ojima H, Kato T, Araki K, et al. Factors predicting long-term responses to splenectomy in patients with idiopathic thrombocytopenic purpura. World J Surg. 2006;30(4):553–559. .
   PubMed Web of Science ®Google Scholar
• Fabris F, Tassan T, Ramon R, et al. Age as the major predictive factor of long-term response to splenectomy in immune thrombocytopenic purpura. Br J Haematol. 2001;112(3):637–640. .
   PubMed Web of Science ®Google Scholar
• Chen X, Peng B, Cai Y, et al. Laparoscopic splenectomy for patients with immune thrombocytopenia and very low platelet count: is platelet transfusion necessary? J Surg Res. 2011;170(2):e225–32. .
   PubMed Web of Science ®Google Scholar
• Zaja F, Barcellini W, Cantoni S, et al. Thrombopoietin receptor agonists for preparing adult patients with immune thrombocytopenia to splenectomy: results of a retrospective, observational GIMEMA study. Am J Hematol. 2016;91(5):E293–5. .
   PubMed Web of Science ®Google Scholar
• Kashiwagi H, Kuwana M, Hato T, et al. Reference guide for management of adult immune thrombocytopenia in Japan: 2019 Revision. Int J Hematol. 2020;111(3):329–351. .
   PubMed Web of Science ®Google Scholar
• Duperier T, Brody F, Felsher J, et al. Predictive factors for successful laparoscopic splenectomy in patients with immune thrombocytopenic purpura. Arch Surg. 2004;139(1):61–66. Chicago, Ill: 1960. discussion 6.
   PubMedGoogle Scholar
• Aramaki M, Matsumoto T, Kitano S. [Laparoscopic and open splenectomy for idiopathic thrombocytopenic purpura]. Nihon Rinsho Japan J Clin Med. 2003;61(4):599–603.
   PubMedGoogle Scholar
• Sotomayor-Ramírez RK. Efficacy and safety of laparoscopic splenectomy: review of 14 adult cases using the lateral approach. Boletin de la Asociacion Medica de Puerto Rico. 2009;101(2):43–49.
   PubMedGoogle Scholar
• Park YH, Yi HG, Kim CS, et al. Clinical Outcome and Predictive Factors in the Response to Splenectomy in Elderly Patients with Primary Immune Thrombocytopenia: a Multicenter Retrospective Study. Acta Haematol. 2016;135(3):162–171. .
   PubMed Web of Science ®Google Scholar
• Vianelli N, Galli M, De Vivo A, et al. Efficacy and safety of splenectomy in immune thrombocytopenic purpura: long-term results of 402 cases. Haematologica. 2005;90(1):72–77.
   PubMed Web of Science ®Google Scholar
• Tada K, Ohta M, Saga K, et al. Long-term outcomes of laparoscopic versus open splenectomy for immune thrombocytopenia. Surg Today. 2018;48(2):180–185. .
   PubMed Web of Science ®Google Scholar
• Gillis S, Dann EJ, Berkman N, et al. Fatal Haemophilus influenzae septicemia following bronchoscopy in a splenectomized patient. Chest. 1993;104(5):1607–1609.
   PubMed Web of Science ®Google Scholar
• Buzelé R, Barbier L, Sauvanet A, et al. Medical complications following splenectomy. J Visc Surg. 2016;153(4):277–286.
   PubMed Web of Science ®Google Scholar
• McCrae KR. Better late than never? Blood. 2013;122(11):1844–1845.
   PubMed Web of Science ®Google Scholar
• Nazi I, Kelton JG, Larché M, et al. The effect of rituximab on vaccine responses in patients with immune thrombocytopenia. Blood. 2013;122(11):1946–1953. .
   PubMed Web of Science ®Google Scholar
• Newland A, Lee EJ, McDonald V, et al. Fostamatinib for persistent/chronic adult immune thrombocytopenia. Immunotherapy. 2018;10(1):9–25.
   PubMed Web of Science ®Google Scholar
• Niscola P, Scaramucci L, Giovannini M. Spleen tyrosine kinase inhibition: a new promising approach to chronic and refractory immune thrombocytopenia. Immunotherapy. 2018;10(1):5–7.
   PubMed Web of Science ®Google Scholar
• Moore DC, Gebru T, Muslimani A. Fostamatinib for the treatment of immune thrombocytopenia in adults. Am J Health-system Pharm. 2019;76(11):789–794.
   PubMed Web of Science ®Google Scholar
• Bussel J, Arnold DM, Grossbard E, et al. Fostamatinib for the treatment of adult persistent and chronic immune thrombocytopenia: results of two phase 3, randomized, placebo-controlled trials. Am J Hematol. 2018;93(7):921–930. .
   PubMed Web of Science ®Google Scholar
• Bussel JB, Arnold DM, Boxer MA, et al. Long-term fostamatinib treatment of adults with immune thrombocytopenia during the phase 3 clinical trial program. Am J Hematol. 2019;94(5):546–553. .
   PubMed Web of Science ®Google Scholar
• Ahn YS, Rocha R, Mylvaganam R, et al. Long-term danazol therapy in autoimmune thrombocytopenia: unmaintained remission and age-dependent response in women. Ann Intern Med. 1989;111(9):723–729.
   PubMed Web of Science ®Google Scholar
• Phillips M. Danazol and idiopathic thrombocytopenic purpura. Ann Intern Med. 1990;112(6):474–475.
   PubMed Web of Science ®Google Scholar
• Liu W, Gu X, Fu R, et al. The Effect of Danazol in Primary Immune Thrombocytopenia: an Analysis of a Large Cohort From a Single Center in China. Clin Appl Thromb Hemost. 2016;22(8):727–733. .
   PubMed Web of Science ®Google Scholar
• Schiavotto C, Castaman G, Rodeghiero F. Treatment of idiopathic thrombocytopenic purpura (ITP) in patients with refractoriness to or with contraindication for corticosteroids and/or splenectomy with immunosuppressive therapy and danazol. Haematologica. 1993;78(6 Suppl 2):29–34.
   PubMedGoogle Scholar
• Rattanathammethee T, Theerajangkhaphichai W, Rattarittamrong E, et al. The Efficacy of Colchicine and Dapsone Combination Therapy in Relapsed Immune Thrombocytopenia. Hematol Rep. 2017;9(1):7034. .
   PubMed Web of Science ®Google Scholar
• Zaja F, Marin L, Chiozzotto M, et al. Dapsone salvage therapy for adult patients with immune thrombocytopenia relapsed or refractory to steroid and rituximab. Am J Hematol. 2012;87(3):321–323.
   PubMed Web of Science ®Google Scholar
• Vancine-Califani SM, De Paula EV, Ozelo MC, et al. Efficacy and safety of dapsone as a second-line treatment in non-splenectomized adults with immune thrombocytopenic purpura. Platelets. 2008;19(7):489–495.
   PubMed Web of Science ®Google Scholar
• Khera S, Pramanik SK, Yanamandra U, et al. Dapsone: an Old but Effective Therapy in Pediatric Refractory Immune Thrombocytopenia. Indian J Hematol Blood Transfus. 2020;36(4):690–694.
   PubMed Web of Science ®Google Scholar
• Sahu KK, Lal A, Mishra AK, et al. Methemoglobinemia in Transplant Recipient Patients: future of Dapsone as Pneumocystis carinii Pneumonia Prophylaxis. Prog Trans. 2020;30(1):71–72. . Aliso Viejo, Calif
   PubMed Web of Science ®Google Scholar
• Molinelli E, Paolinelli M, Campanati A, et al. Metabolic, pharmacokinetic, and toxicological issues surrounding dapsone. Expert Opin Drug Metab Toxicol. 2019;15(5):367–379.
   PubMed Web of Science ®Google Scholar
• Chang H, Tang TC, Hung YS, et al. Immune thrombocytopenia: effectiveness of frontline steroids and comparison of azathioprine, splenectomy, and rituximab as second-line treatment. Eur J Haematol. 2018;101(4):549–555. .
   PubMed Web of Science ®Google Scholar
• Yenson PR, Forrest D, Schmiegelow K, et al. Azathioprine-associated acute myeloid leukemia in a patient with Crohn’s disease and thiopurine S-methyltransferase deficiency. Am J Hematol. 2008;83(1):80–83.
   PubMed Web of Science ®Google Scholar
• Staatz CE, Tett SE. Pharmacology and toxicology of mycophenolate in organ transplant recipients: an update. Arch Toxicol. 2014;88(7):1351–1389.
   PubMed Web of Science ®Google Scholar
• Taylor A, Neave L, Solanki S, et al. Mycophenolate mofetil therapy for severe immune thrombocytopenia. Br J Haematol. 2015;171(4):625–630. .
   PubMed Web of Science ®Google Scholar
• Colović M, Suvajdzic N, Colović N, et al. Mycophenolate mophetil therapy for chronic immune thrombocytopenic purpura resistant to steroids, immunosuppressants, and/or splenectomy in adults. Platelets. 2011;22(2):153–156.
   PubMed Web of Science ®Google Scholar
• Hou M, Peng J, Shi Y, et al. Mycophenolate mofetil (MMF) for the treatment of steroid-resistant idiopathic thrombocytopenic purpura. Eur J Haematol. 2003;70(6):353–357. .
   PubMed Web of Science ®Google Scholar
• Emilia G, Messora C, Longo G, et al. Long-term salvage treatment by cyclosporin in refractory autoimmune haematological disorders. Br J Haematol. 1996;93(2):341–344.
   PubMed Web of Science ®Google Scholar
• Emilia G, Morselli M, Luppi M, et al. Long-term salvage therapy with cyclosporin A in refractory idiopathic thrombocytopenic purpura. Blood. 2002;99(4):1482–1485. .
   PubMed Web of Science ®Google Scholar
• 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.
   PubMed Web of Science ®Google Scholar
• Verlin M, Laros RK Jr., Penner JA. Treatment of refractory thrombocytopenic purpura with cyclophosphamine. Am J Hematol. 1976;1(1):97–104.
   PubMed Web of Science ®Google Scholar
• Reiner A, Gernsheimer T, Slichter SJ. Pulse cyclophosphamide therapy for refractory autoimmune thrombocytopenic purpura. Blood. 1995;85(2):351–358.
   PubMed Web of Science ®Google Scholar
• Reid DM, Shulman NR. Pulse cyclophosphamide to treat idiopathic thrombocytopenic purpura. Blood. 1995;86(1):414–415.
   PubMed Web of Science ®Google Scholar
• Krause JR. Chronic idiopathic thrombocytopenic purpura (ITP:) development of acute non-lymphocytic leukemia subsequent to treatment with cyclophosphamide. Med Pediatr Oncol. 1982;10(1):61–65.
   PubMedGoogle Scholar
• Sahu KK, Prakash G, Khadwal A, et al. Case of Hemorrhagic Cystitis in Allogeneic Hematopoietic Stem Cell Transplant Patient. Indian J Hematol Blood Transfus. 2016;32(Suppl 1):196–200.
   PubMedGoogle Scholar
• Stirnemann J, Kaddouri N, Khellaf M, et al. Vincristine efficacy and safety in treating immune thrombocytopenia: a retrospective study of 35 patients. Eur J Haematol. 2016;96(3):269–275. .
   PubMed Web of Science ®Google Scholar
• Park YH, Yi HG, Lee MH, et al. Clinical efficacy and tolerability of vincristine in splenectomized patients with refractory or relapsed immune thrombocytopenia: a retrospective single-center study. Int J Hematol. 2016;103(2):180–188.
   PubMed Web of Science ®Google Scholar
• Shvidel L, Sigler E, Shtalrid M, et al. Vincristine-loaded platelet infusion for treatment of refractory autoimmune hemolytic anemia and chronic immune thrombocytopenia: rethinking old cures. Am J Hematol. 2006;81(6):423–425.
   PubMed Web of Science ®Google Scholar
• Zhou H, Xu M, Qin P, et al. A multicenter randomized open-label study of rituximab plus rhTPO vs rituximab in corticosteroid-resistant or relapsed ITP. Blood. 2015;125(10):1541–1547. .
   PubMed Web of Science ®Google Scholar
• Arai Y, Jo T, Matsui H, et al. Comparison of up-front treatments for newly diagnosed immune thrombocytopenia -a systematic review and network meta-analysis. Haematologica. 2018;103(1):163–171.
   PubMed Web of Science ®Google Scholar
• Choi PY, Roncolato F, Badoux X, et al. A novel triple therapy for ITP using high-dose dexamethasone, low-dose rituximab, and cyclosporine (TT4). Blood. 2015;126(4):500–503.
   PubMed Web of Science ®Google Scholar
• Arnold DM, Nazi I, Santos A, et al. Combination immunosuppressant therapy for patients with chronic refractory immune thrombocytopenic purpura. Blood. 2010;115(1):29–31. .
   PubMed Web of Science ®Google Scholar
• Figueroa M, Gehlsen J, Hammond D, et al. Combination chemotherapy in refractory immune thrombocytopenic purpura. N Engl J Med. 1993;328(17):1226–1229. .
   PubMed Web of Science ®Google Scholar
• Gudbrandsdottir S, Birgens HS, Frederiksen H, et al. Rituximab and dexamethasone vs dexamethasone monotherapy in newly diagnosed patients with primary immune thrombocytopenia. Blood. 2013;121(11):1976–1981. .
   PubMed Web of Science ®Google Scholar
• Zaja F, Baccarani M, Mazza P, et al. Dexamethasone plus rituximab yields higher sustained response rates than dexamethasone monotherapy in adults with primary immune thrombocytopenia. Blood. 2010;115(14):2755–2762. .
   PubMed Web of Science ®Google Scholar
• 3Boruchov DM, Gururangan S, Driscoll MC, et al. Multiagent induction and maintenance therapy for patients with refractory immune thrombocytopenic purpura (ITP). Blood. 2007;110(10):3526–3531.
   PubMed Web of Science ®Google Scholar
• Nampoothiri RV, Singh C, Mishra K, et al. Immune thrombocytopenia is the commonest diagnosis on consultative hematology - a single centre experience. Indian J Hematol Blood Transfus. 2017 Nov; 33(Suppl 1):S104
   Google Scholar
• Goel R, Chopra S, Tobian AAR, et al. Platelet transfusion practices in immune thrombocytopenia related hospitalizations. Transfusion. 2019;59(1):169–176. .
   PubMed Web of Science ®Google Scholar
• Estcourt LJ, Birchall J, Allard S, et al. Guidelines for the use of platelet transfusions. Br J Haematol. 2017;176(3):365–394. .
   PubMed Web of Science ®Google Scholar
• Zhang MK, Xu TQ, Zhang XJ, et al. Thrombocytopenia in 737 adult intensive care unit patients: a real-world study of associated factors, drugs, platelet transfusion, and clinical outcome. SAGE Open Med. 2020;8:2050312120958908.
   Web of Science ®Google Scholar
• Goel R, Ness PM, Takemoto CM, Goel R, Ness PM, Takemoto CM, Krishnamurti L, King KE, Tobian AA. Platelet transfusions in platelet consumptive disorders are associated with arterial thrombosis and in-hospital mortality. Blood. 2015;125(9):1470–1476. .
   PubMed Web of Science ®Google Scholar
• Wardrop D, Estcourt LJ, Brunskill SJ, et al. Antifibrinolytics (lysine analogues) for the prevention of bleeding in patients with haematological disorders. Cochrane Database Syst Rev. 2013;(7):Cd009733. DOI:10.1002/14651858.CD009733.pub2.
   PubMed Web of Science ®Google Scholar
• Estcourt LJ, Desborough M, Brunskill SJ, et al. Antifibrinolytics (lysine analogues) for the prevention of bleeding in people with haematological disorders. Cochrane Database Syst Rev. 2016;3(3):Cd009733. .
   PubMedGoogle Scholar
• Lethaby A, Hussain M, Rishworth JR, et al. Progesterone or progestogen-releasing intrauterine systems for heavy menstrual bleeding. Cochrane Database Syst Rev. 2015;4(6):Cd002126.
   PubMed Web of Science ®Google Scholar
• Bofill Rodriguez M, Lethaby A, Low C, et al. Cyclical progestogens for heavy menstrual bleeding. Cochrane Database Syst Rev. 2019;8(8):Cd001016.
   PubMedGoogle Scholar
• Eslick R, McLintock C. Managing ITP and thrombocytopenia in pregnancy. Platelets. 2020;31(3):300–306.
   PubMed Web of Science ®Google Scholar
• Smith B, Kiessling A, Lledo-Garcia R, et al. Generation and characterization of a high affinity anti-human FcRn antibody, rozanolixizumab, and the effects of different molecular formats on the reduction of plasma IgG concentration. mAbs. 2018;10(7):1111–1130. .
   PubMed Web of Science ®Google Scholar
• Dou X, Yang R. Current and emerging treatments for immune thrombocytopenia. Expert Rev Hematol. 2019;12(9):723–732.
   PubMed Web of Science ®Google Scholar