Skip to main content
SpringerLink
Log in

Long-term follow-up of idiopathic thrombotic thrombocytopenic purpura treated with rituximab

  • Original Article
  • Published:
Annals of Hematology Aims and scope Submit manuscript

Abstract

Rituximab may be used to treat patients with thrombotic thrombocytopenic purpura (TTP) refractory to plasma exchange or recurrent disease. While initial response rates are reported to be high, long-term follow-up data of patients treated with rituximab are not available to date, however important to estimate the safety and benefit of this treatment. Twelve patients with non-familial idiopathic TTP refractory to plasma exchange or with recurrent disease treated with rituximab between 2000 and 2008 were reexamined. The median follow-up was 49.6 months, ranging from 11 to 97 months. All patients achieved initial complete remission after application of rituximab. During follow-up, nine patients remained disease-free and three patients suffered from recurrent disease. All patients with recurrent disease responded to subsequent rituximab therapy. No long-term side effects were noted during the follow-up period. In conclusion, rituximab represents an effective second-line treatment option in relapsing or refractory TTP. Still, patients need to be closely monitored for relapses with extended follow-up.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Veyradier A, Meyer D (2005) Thrombotic thrombocytopenic purpura and its diagnosis. J Thromb Haemost 3(11):2420–2427

    Article  CAS  PubMed  Google Scholar 

  2. Moake JL, Rudy CK, Troll JH, Weinstein MJ, Colannino NM, Azocar J, Seder RH, Hong SL, Deykin D (1982) Unusually large plasma factor VIII: von Willebrand factor multimers in chronic relapsing thrombotic thrombocytopenic purpura. N Engl J Med 307(23):1432–1435

    Article  CAS  PubMed  Google Scholar 

  3. Tsai HM (1996) Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion. Blood 87(10):4235–4244

    CAS  PubMed  Google Scholar 

  4. Furlan M, Robles R, Lammle B (1996) Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. Blood 87(10):4223–4234

    CAS  PubMed  Google Scholar 

  5. Furlan M, Robles R, Solenthaler M, Wassmer M, Sandoz P, Lammle B (1997) Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura. Blood 89(9):3097–3103

    CAS  PubMed  Google Scholar 

  6. Levy GG, Nichols WC, Lian EC, Foroud T, McClintick JN, McGee BM, Yang AY, Siemieniak DR, Stark KR, Gruppo R et al (2001) Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature 413(6855):488–494

    Article  CAS  PubMed  Google Scholar 

  7. Tsai HM, Lian EC (1998) Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med 339(22):1585–1594

    Article  CAS  PubMed  Google Scholar 

  8. Furlan M, Robles R, Galbusera M, Remuzzi G, Kyrle PA, Brenner B, Krause M, Scharrer I, Aumann V, Mittler U et al (1998) von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic–uremic syndrome. N Engl J Med 339(22):1578–1584

    Article  CAS  PubMed  Google Scholar 

  9. 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(6):398–403

    Article  CAS  PubMed  Google Scholar 

  10. 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(6):393–397

    Article  CAS  PubMed  Google Scholar 

  11. Sadler JE (2008) Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura. Blood 112(1):11–18

    Article  CAS  PubMed  Google Scholar 

  12. Kremer Hovinga JA, Meyer SC (2008) Current management of thrombotic thrombocytopenic purpura. Curr Opin Hematol 15(5):445–450

    Article  PubMed  Google Scholar 

  13. George JN (2000) How I treat patients with thrombotic thrombocytopenic purpura–hemolytic uremic syndrome. Blood 96(4):1223–1229

    CAS  PubMed  Google Scholar 

  14. George JN, Sadler JE, Lammle B (2002) Platelets: thrombotic thrombocytopenic purpura. Hematology Am Soc Hematol Educ Program, pp 315–334

  15. Vesely SK, George JN, Lammle B, Studt JD, Alberio L, El-Harake MA, Raskob GE (2003) ADAMTS13 activity in thrombotic thrombocytopenic purpura–hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients. Blood 102(1):60–68

    Article  CAS  PubMed  Google Scholar 

  16. Chemnitz J, Draube A, Scheid C, Staib P, Schulz A, Diehl V, Sohngen D (2002) Successful treatment of severe thrombotic thrombocytopenic purpura with the monoclonal antibody rituximab. Am J Hematol 71(2):105–108

    Article  CAS  PubMed  Google Scholar 

  17. Fakhouri F, Vernant JP, Veyradier A, Wolf M, Kaplanski G, Binaut R, Rieger M, Scheiflinger F, Poullin P, Deroure B et al (2005) Efficiency of curative and prophylactic treatment with rituximab in ADAMTS13-deficient thrombotic thrombocytopenic purpura: a study of 11 cases. Blood 106(6):1932–1937

    Article  CAS  PubMed  Google Scholar 

  18. Scully M, Cohen H, Cavenagh J, Benjamin S, Starke R, Killick S, Mackie I, Machin SJ (2007) Remission in acute refractory and relapsing thrombotic thrombocytopenic purpura following rituximab is associated with a reduction in IgG antibodies to ADAMTS-13. Br J Haematol 136(3):451–461

    Article  CAS  PubMed  Google Scholar 

  19. Heidel F, Lipka DB, von Auer C, Huber C, Scharrer I, Hess G (2007) Addition of rituximab to standard therapy improves response rate and progression-free survival in relapsed or refractory thrombotic thrombocytopenic purpura and autoimmune haemolytic anaemia. Thromb Haemost 97(2):228–233

    CAS  PubMed  Google Scholar 

  20. Ling HT, Field JJ, Blinder MA (2009) Sustained response with rituximab in patients with thrombotic thrombocytopenic purpura: a report of 13 cases and review of the literature. Am J Hematol 84(7):418–421

    Article  CAS  PubMed  Google Scholar 

  21. Bresin E, Gastoldi S, Daina E, Belotti D, Pogliani E, Perseghin P, Scalzulli PR, Paolini R, Marceno R, Remuzzi G, Galbusera M (2009) Rituximab as pre-emptive treatment in patients with thrombotic thrombocytopenic purpura and evidence of anti-ADAMTS13 autoantibodies. Thromb Haemost 101:233–238

    CAS  PubMed  Google Scholar 

  22. Cooper N, Arnold DM. (2010) The effect of rituximab on humoral and cell mediated immunity and infection in the treatment of autoimmune diseases. Br J Haematol 149(1):3–13(11)

    Article  CAS  PubMed  Google Scholar 

  23. Kappers-Klunne MC, Wijermans P, Fijnheer R, Croockewit AJ, van der Holt B, de Wolf JTM, Löwenberg B, Brand A (2005) Splenectomy for the treatment of thrombotic thrombocytopenic purpura. Br J Haematol 130:768–776

    Article  CAS  PubMed  Google Scholar 

  24. Garvey B (2008) Rituximab in the treatment of autoimmune haematological disorders. Br J Haematol 141(2):149–169

    Article  CAS  PubMed  Google Scholar 

  25. George JN, Woodson RD, Kiss JE, Kojouri K, Vesely SK (2006) Rituximab therapy for thrombotic thrombocytopenic purpura: a proposed study of the Transfusion Medicine/Hemostasis Clinical Trials Network with a systematic review of rituximab therapy for immune-mediated disorders. J Clin Apher 21(1):49–56

    Article  PubMed  Google Scholar 

Download references

Authorship and disclosures

JMC recruited the patients and wrote the paper, JU recruited the patients, and MH discussed the results. CS wrote the paper and takes the main responsibility for the paper. The authors reported no potential conflicts of interest.

Author information

Authors and Affiliations

  1. Department I of Internal Medicine, University of Cologne, Cologne, Germany

    Jens Marcus Chemnitz, Jens Uener, Michael Hallek & Christof Scheid

  2. Department I of Internal Medicine, University Hospital of Cologne, Kerpener Strasse 62, 50937, Cologne, Germany

    Jens Marcus Chemnitz

Authors
  1. Jens Marcus Chemnitz
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Jens Uener
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Michael Hallek
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Christof Scheid
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Jens Marcus Chemnitz.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Chemnitz, J.M., Uener, J., Hallek, M. et al. Long-term follow-up of idiopathic thrombotic thrombocytopenic purpura treated with rituximab. Ann Hematol 89, 1029–1033 (2010). https://doi.org/10.1007/s00277-010-0968-3

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00277-010-0968-3

Keywords

Search

Navigation