Rituximab Provided Long-Term Remission in a Patient with Refractory Relapsing Thrombotic Thrombocytopenic Purpura

  • Satoru Kosugi
  • Masanori Matsumoto
  • Yasushi Ohtani
  • Hironori Take
  • Hiromichi Ishizashi
  • Yoshihiro Fujimura
  • Jun Kuyama
Case Report


We describe a 69-year-old man with refractory relapsing thrombotic thrombocytopenic purpura (TTP) successfully treated with rituximab. The patient had once been successfully treated with plasmapheresis and vincristine, but he had relapsed after a short period. Although plasmapheresis, vincristine, and splenectomy could not achieve a consistent elevation of the platelet count, rituximab administration provided sustained remission for more than 7 months. Rituximab should be considered as a therapeutic alternative for refractory TTP.

Key words

Thrombotic thrombocytopenic purpura (TTP) Rituximab von Willebrand factor-cleaving protease (VWF-CP) Anti-ADAMTS13 autoantibody 


  1. 1.
    Bukowski RM. Thrombotic thrombocytopenic purpura: a review. Prog Hemost Thromb. 1982;6:287–337.PubMedGoogle Scholar
  2. 2.
    Furlan M, Robles R, Galbusera M, et al. von Willebrand factor- cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med. 1998;339:1578–1584.PubMedCrossRefGoogle Scholar
  3. 3.
    Furlan M, Robles R, Solenthaler M, Wassmer M, Sandoz P, Lammle B. Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura. Blood. 1997;89:3097–3103.PubMedGoogle Scholar
  4. 4.
    Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med. 1998;339:1585–1594.PubMedCrossRefGoogle Scholar
  5. 5.
    Rock GA, Shumak KH, Buskard NA, et al. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura: Canadian Apheresis Study Group. N Engl J Med. 1991;325:393–397.PubMedGoogle Scholar
  6. 6.
    Shumak KH, Rock GA, Nair RC. Late relapse in patients successfully treated for thrombotic thrombocytopenic purpura: Canadian Apheresis Group. Ann Intern Med. 1995;44:2836–2840.Google Scholar
  7. 7.
    Bell WR. Thrombotic thrombocytopenic purpura/hemolytic uremic syndrome relapse: frequency, pathogenesis, and meaning. Semin Hematol. 1997;34:134–139.PubMedGoogle Scholar
  8. 8.
    Kuwaan HC, Soff GA. Management of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Semin Hematol. 1997;34:159–166.Google Scholar
  9. 9.
    Allford SL, Hunt BJ, Rose P, Machin SJ. Guidelines on the diagnosis and management of the thrombotic microangiopathic haemolytic anaemias. Br J Haematol. 2003;120:556–573.PubMedCrossRefGoogle Scholar
  10. 10.
    Aqui NA, Stein SH, Konkle BA, Abrams CS, Strobl FJ. Role of splenectomy in patients with refractory or relapsed thrombotic thrombocytopenic purpura. J Clin Apheresis. 2003;18:51–54.PubMedCrossRefGoogle Scholar
  11. 11.
    Hovinga JAK, Studt JD, Biasiutti FD, et al. Splenectomy in relapsing and plasma-refractory acquired thrombotic thrombocytopenic purpura. Br J Haematol. 2004;89:320–324.Google Scholar
  12. 12.
    Boye J, Elter T, Engert A. An overview of the current clinical use of the anti-CD20 monoclonal antibody rituximab. Ann Oncol. 2003;14:520–535.PubMedCrossRefGoogle Scholar
  13. 13.
    Levine TD, Pestronk A. IgM antibody-related polyneuropathies: B-cell depletion chemotherapy using rituximab. Neurology. 1999; 52:1701–1704.PubMedGoogle Scholar
  14. 14.
    Stasi R, Pagano A, Stipa E, Amadori S. Rituximab chimeric anti- CD20 monoclonal antibody treatment for adults with chronic idio- pathic thrombocytopenic purpura. Blood. 2001;98:952–957.PubMedCrossRefGoogle Scholar
  15. 15.
    Remmuzzi G, Chiuruchiu C, Abbate M,Brusegan V, Bontempelli M, Ruggenenti P. Rituximab for idiopathic membranous nephropathy. Lancet. 2002;360:923–924.CrossRefGoogle Scholar
  16. 16.
    Gutterman LA, Kloster B, Tsai HM. Rituximab therapy for refractory thrombotic thrombocytopenic purpura. Blood Cells Mol Dis. 2002;28:385–391.PubMedCrossRefGoogle Scholar
  17. 17.
    Chemnitz J, Draube A, Scheid C, et al. Successful treatment of severe thrombotic thrombocytopenic purpura with the monoclonal antibody rituximab. Am J Hematol. 2002;71:105–108.PubMedCrossRefGoogle Scholar
  18. 18.
    Zheng X, Pallera AM, Goodnough LT, Sadler JE, Blinder MA. Remission of chronic thrombotic thrombocytopenic purpura after treatment with cyclophosphamide and rituximab. Ann Intern Med. 2003;138:105–108.PubMedGoogle Scholar
  19. 19.
    Tsai HM, Shulman K. Rituximab induces remission of cerebral ischemia caused by thrombotic thrombocytopenic purpura. Eur J Haematol. 2003;70:183–185.PubMedCrossRefGoogle Scholar
  20. 20.
    Yomtovian R, Niklinski W, Silver B, Sarode R, Tsai HM. Rituximab for chronic recurring thrombotic thrombocytopenic purpura: a case report and review of the literature. Br J Haematol. 2004;124:787–795.PubMedCrossRefGoogle Scholar
  21. 21.
    Stein YG, Aeidman A, Fradin Z, Varon M, Cohen A, Mittelman M. Treatment of resistant thrombotic thrombocytopenic purpura with rituximab and cyclophosphamide. Int J Hematol. 2004;80:94–96.PubMedCrossRefGoogle Scholar
  22. 22.
    Sugio Y, Okamura T, Shimoda K, et al. Ticlopidine-associated thrombotic thrombocytopenic purpura with an IgG-type inhibitor to von Willebrand factor-cleaving protease activity. Int J Hematol. 2001;74:347–351.PubMedCrossRefGoogle Scholar
  23. 23.
    Kawahara M, Kanno M, Matsumoto M, Nakamura S, Fujimura Y, Ueno S. Diffuse neurodeficits in intravascular lymphomatosis with ADAMTS13 inhibitor. Neurology. 2004;63:1731–1733.PubMedGoogle Scholar

Copyright information

© The Japanese Society of Hematology 2005

Authors and Affiliations

  • Satoru Kosugi
    • 1
  • Masanori Matsumoto
    • 2
  • Yasushi Ohtani
    • 1
  • Hironori Take
    • 1
  • Hiromichi Ishizashi
    • 2
  • Yoshihiro Fujimura
    • 2
  • Jun Kuyama
    • 1
  1. 1.Department of Internal MedicineToyonaka Municipal HospitalOsakaJapan
  2. 2.Department of Blood Transfusion MedicineNara Medical UniversityNaraJapan

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