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High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: a randomized study

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Abstract

Therapeutic plasma exchange (PE) is the accepted therapy for thrombotic thrombocytopenic purpura (TTP). Because not all patients achieve remission, other treatment modalities have been used in addition to PE, but no randomized clinical trial evaluated their efficacy. The aim of this multicentric study was to compare the effectiveness of standard- versus high-dose methylprednisolone as an adjunctive treatment to PE in the acute phase of TTP. Sixty patients with idiopathic TTP were randomized to receive methylprednisolone 1 mg/kg/die intravenous or 10 mg/kg/die for 3 days, thereafter, 2.5 mg/kg/die in addition to PE. Both dosages of steroids were well tolerated. At the end of induction therapy (day 23), the percentage of patients failing to achieve complete remission was significantly higher in the standard dose (16 of 30) than in the high-dose group (seven of 30). Also, the number of cases without a good response at day 9 and the number of deaths were higher in the standard-dose arm, but the differences did not reach the statistical significance. Results of present study indicate that the association of PE with high-dose instead of standard-dose steroids reduces the percentage of TTP patients that fail to achieve complete remission.

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References

  1. Bukowski RM, King JW, Hewlett JS (1977) Plasmapheresis in the treatment of thrombotic thrombocytopenic purpura. Blood 50:413–417

    CAS  PubMed  Google Scholar 

  2. Byrnes JJ, Khurana M (1977) Treatment of thrombotic thrombocytopenic purpura with plasma. N Engl J Med 297:1386–1389

    CAS  PubMed  Google Scholar 

  3. Rock GA, Shumak KH, Buskard NA, Blanchette VS, Kelton JG, Nair RC, Spasoff RA (1991) Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group. N Engl J Med 325:393–397

    CAS  PubMed  Google Scholar 

  4. Furlan M, Robles R, Galbusera M, Remuzzi G, Kyrle PA, Brenner B, Krause M, Scharrer I, Aumann V, Mittler U, Solenthaler M, Lammle B (1998) von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med 339:1578–1584

    Article  CAS  PubMed  Google Scholar 

  5. George JN (2006) Clinical practice. Thrombotic thrombocytopenic purpura. N Engl J Med 354:1927–1935

    Article  CAS  PubMed  Google Scholar 

  6. Allford SL, Hunt BJ, Rose P, Machin SJ, Haemostasis and Thrombosis Task Force, British Committee for Standards in Haematology (2003) Guidelines on the diagnosis and management of the thrombotic microangiopathic haemolytic anaemias. Br J Haematol 120:556–573

    Article  PubMed  Google Scholar 

  7. Rock G, Shumak KH, Sutton DM, Buskard NA, Nair RC (1996) Cryosupernatant as replacement fluid for plasma exchange in thrombotic thrombocytopenic purpura. Br J Haematol 94:383–386

    Article  CAS  PubMed  Google Scholar 

  8. Rock G, Anderson D, Clark W, Leblond P, Palmer D, Sternbach M, Sutton D, Wells G (2005) Does cryosupernatant plasma improve outcome in thrombotic thrombocytopenic purpura? No answer yet. Br J Haematol 129:79–86

    Article  CAS  PubMed  Google Scholar 

  9. Bobbio-Pallavicini E, Gugliotta L, Centurioni R, Porta C, Vianelli N, Billio A, Tacconi F, Ascari E (1997) Antiplatelet agents in thrombotic thrombocytopenic purpura (TTP). Results of a randomized multicenter trial by the Italian Cooperative Group for TTP. Haematologica 82:429–435

    CAS  PubMed  Google Scholar 

  10. Mintz PD, Neff A, MacKenzie M, Goodnough LT, Hillyer C, Kessler C, McCrae K, Menitove JE, Skikne BS, Damon L, Lopez-Plaza I, Rouault C, Crookston KP, Benjamin RJ, George J, Lin JS, Corash L, Conlan MG (2006) A randomized, controlled phase III trial of therapeutic plasma exchange with fresh-frozen plasma (FFP) prepared with amotosalen and ultraviolet A light compared to untreated FFP in thrombotic thrombocytopenic purpura. Transfusion 46:1693–1704

    Article  CAS  PubMed  Google Scholar 

  11. Cataland SR, Jin M, Ferketich AK, Kennedy MS, Kraut EH, George JN, Wu HM (2007) An evaluation of cyclosporin and corticosteroids individually as adjuncts to plasma exchange in the treatment of thrombotic thrombocytopenic purpura. Br J Haematol 136:146–149

    Article  CAS  PubMed  Google Scholar 

  12. Bell WR, Braine HG, Ness PM, Kickler TS (1991) Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Clinical experience in 108 patients. N Engl J Med 325:398–403

    Article  CAS  PubMed  Google Scholar 

  13. Rojnuckarin P, Watanaboonyongcharoen P, Akkawat B, Intragumtornchai T (2006) The role of pulse dexamethasone in acquired idiopathic thrombotic thrombocytopenic purpura. J Thromb Haemost 4:1148–1150

    Article  CAS  PubMed  Google Scholar 

  14. Toyoshige M, Zaitsu Y, Okafuji K, Inoue Y, Hiroshige Y, Matsumoto N, Kaku K, Kaneko T (1992) Successful treatment of thrombotic thrombocytopenic purpura with high-dose corticosteroid. Am J Hematol 41:69

    Article  CAS  PubMed  Google Scholar 

  15. Shariatmadar S, Nassiri M, Vincek V (2005) Effect of plasma exchange on cytokines measured by multianalyte bead array in thrombotic thrombocytopenic purpura. Am J Hematol 79:83–88

    Article  CAS  PubMed  Google Scholar 

  16. Cataland SR, Jin M, Lin S, Kennedy MS, Kraut EH, George JN, Wu HM (2007) Cyclosporin and plasma exchange in thrombotic thrombocytopenic purpura: long-term follow-up with serial analysis of ADAMTS13 activity. Br J Haematol 139:486–493

    Article  CAS  PubMed  Google Scholar 

  17. Mori Y, Wada H, Gabazza EC, Minami N, Nobori T, Shiku H, Yagi H, Ishizashi H, Matsumoto M, Fujimura Y (2002) Predicting response to plasma exchange in patients with thrombotic thrombocytopenic purpura with measurement of vWF-cleaving protease activity. Transfusion 42:572–580

    Article  CAS  PubMed  Google Scholar 

  18. Coppo P, Bengoufa D, Veyradier A, Wolf M, Bussel A, Millot GA, Malot S, Heshmati F, Mira JP, Boulanger E, Galicier L, Durey-Dragon MA, Frémeaux-Bacchi V, Ramakers M, Pruna A, Bordessoule D, Gouilleux V, Scrobohaci ML, Vernant JP, Moreau D, Azoulay E, Schlemmer B, Guillevin L, Lassoued K (2004) Severe ADAMTS13 deficiency in adult idiopathic thrombotic microangiopathies defines a subset of patients characterized by various autoimmune manifestations, lower platelet count, and mild renal involvement. Medicine (Baltimore) 83:233–244

    Article  CAS  Google Scholar 

  19. Coppo P, Bengoufa D, Veyradier A, Wolf M, Bussel A, Millot GA, Malot S, Heshmati F, Mira JP, Boulanger E, Galicier L, Durey-Dragon MA, Frémeaux-Bacchi V, Ramakers M, Pruna A, Bordessoule D, Gouilleux V, Scrobohaci ML, Vernant JP, Moreau D, Azoulay E, Schlemmer B, Guillevin L, Lassoued K (2005) Prognostic value of inhibitory anti-ADAMTS13 antibodies in adult-acquired thrombotic thrombocytopenic purpura. Br J Haematol 132:66–74

    Article  CAS  Google Scholar 

  20. Böhm M, Betz C, Miesbach W, Krause M, von Auer C, Geiger H, Scharrer I (2005) The course of ADAMTS-13 activity and inhibitor titre in the treatment of thrombotic thrombocytopenic purpura with plasma exchange and vincristine. Br J Haematol 129:644–652

    Article  PubMed  CAS  Google Scholar 

  21. Mannucci PM, Peyvandi F (2007) TTP and ADAMTS13: when is testing appropriate? Hematology Am Soc Hematol Educ Program 121–126

  22. Ferrari S, Scheiflinger F, Rieger M, Mudde G, Wolf M, Coppo P, Girma JP, Azoulay E, Brun-Buisson C, Fakhouri F, Mira JP, Oksenhendler E, Poullin P, Rondeau E, Schleinitz N, Schlemmer B, Teboul JL, Vanhille P, Vernant JP, Meyer D, Veyradier A (2007) Prognostic value of anti-ADAMTS 13 antibody features (Ig isotype, titer, and inhibitory effect) in a cohort of 35 adult French patients undergoing a first episode of thrombotic microangiopathy with undetectable ADAMTS 13 activity. Blood 109:2815–2822

    CAS  PubMed  Google Scholar 

  23. Jin M, Casper TC, Cataland SR, Kennedy MS, Lin S, Li YJ, Wu HM (2008) Relationship between ADAMTS13 activity in clinical remission and the risk of TTP relapse. Br J Haematol 141:651–658

    Article  CAS  PubMed  Google Scholar 

  24. Peyvandi F, Lavoretano S, Palla R, Feys HB, Vanhoorelbeke K, Battaglioli T, Valsecchi C, Canciani MT, Fabris F, Zver S, Réti M, Mikovic D, Karimi M, Giuffrida G, Laurenti L, Mannucci PM (2008) ADAMTS13 and anti-ADAMTS13 antibodies as markers for recurrence of acquired thrombotic thrombocytopenic purpura during remission. Haematologica 93:232–239

    Article  CAS  PubMed  Google Scholar 

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Correspondence to Carlo L. Balduini.

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Balduini, C.L., Gugliotta, L., Luppi, M. et al. High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: a randomized study. Ann Hematol 89, 591–596 (2010). https://doi.org/10.1007/s00277-009-0877-5

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  • DOI: https://doi.org/10.1007/s00277-009-0877-5

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