|
|
Blood, 15 September 2005, Vol. 106, No. 6, pp. 1932-1937.
Prepublished online as a Blood First Edition Paper on June 2, 2005; DOI 10.1182/blood-2005-03-0848.
 Previous Article | Next Article 
Submitted March 1, 2005
Accepted May 16, 2005
Efficiency of curative and prophylactic treatment with rituximab in ADAMTS13 deficient-thrombotic thrombocytopenic purpura: a study of 11 cases
Fadi Fakhouri*, Jean-Paul Vernant, Agnes Veyradier, Martine Wolf, Gilles Kaplanski, Raynald Binaut, Manfred Rieger, Friedrich Scheiflinger, Pascale Poullin, Benjamin Deroure, Richard Delarue, Philippe Lesavre, Philippe Vanhille, Olivier Hermine, Giuseppe Remuzzi, and Jean-Pierre Grunfeld
Service de Nephrologie, Hopital Necker, AP-HP, Universite Paris V-Rene Descartes, Paris, France
Service d'Hematologie, Hopital de La Pitie-Salpetriere, AP-HP, Paris, France
Service d'Hematologie biologique, Hopital Antoine Beclere, AP-HP, Clamart, France; INSERM U143, Le Kremlin Bicetre, France
Service d'Hematologie biologique, Hopital Antoine Beclere, AP-HP, Clamart, France
Service de Medecine interne, Hopital de La Conception, Marseille, France
Service de Medecine interne et de Nephrologie, Hopital de Valenciennes, Valenciennes, France
Baxter Biomedical Research Center, Orth/Donau, Austria
Service d'Hemapherese et Autotransfusion, Hopital de La Conception, Marseille, France
Service d'Hematologie, Hopital Necker-Enfants Malades, AP-HP, Paris, France
Service de Nephrologie, Hopital Necker, AP-HP, Universite Paris V-Rene Descartes, Paris, France; INSERM U507, Paris, France
Mario Negri Institute for Pharmacological Research, Bergamo, Italy
* Corresponding author; email: fadi.fakhouri{at}nck.ap-hop-paris.fr.
Thrombotic thrombocytopenic purpura (TTP) is a life threatening disease occurring mainly in young adults. Acquired cases are usually due to antibodies directed against ADAMTS13, a protease that cleaves the von Willebrand factor multimers. Prognosis has been improved by plasmatherapy but some acute severe forms are refractory to this treatment and acheiving a sustained remission is still a challenge in chronic relapsing forms.
We therefore conducted a multicentric open-label prospective trial in order to test the efficacy of rituximab, an anti-B cell monoclonal antibody, as a curative and prophylactic treatment in patients with TTP due to anti-ADAMTS13 antibodies.
Six patients were included during an acute refractory TTP episode. Five patients with severe relapsing TTP and persistent anti-ADAMTS13 antibodies were prophylactically treated during remission. All patients received 4 weekly infusions of rituximab. The target of treatment was to restore a significant ADAMTS13 plasma activity ( > 10%). Treatment with rituximab led to clinical remission in all cases of acute refractory TTP. In all patients, anti-ADAMTS13 antibodies disappeared and a significant (18-75%) plasma ADAMTS13 activity was detected following treatment. Tolerance of rituximab was good.
Rituximab is a promising first-line immunosuppressive treatment in patients with acute refractory and severe relapsing TTP related to anti-ADAMTS13 antibodies.
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
Related Article in Blood Online:
-
Controlling chronic TTP
- James N. George
Blood 2005 106: 1896.
[Full Text]
[PDF]
This article has been cited by other articles:
|
|
|
|
 
B. Godeau, R. Porcher, O. Fain, F. Lefrere, P. Fenaux, S. Cheze, A. Vekhoff, M.-P. Chauveheid, J. Stirnemann, L. Galicier, et al.
Rituximab efficacy and safety in adult splenectomy candidates with chronic immune thrombocytopenic purpura: results of a prospective multicenter phase 2 study
Blood,
August 15, 2008;
112(4):
999 - 1004.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
N. Limal, P. Cacoub, D. Sene, I. Guichard, and J.-C. Piette
Letter to the Editor: Rituximab for the treatment of thrombotic thrombocytopenic purpura in systemic lupus erythematosus
Lupus,
January 1, 2008;
17(1):
69 - 71.
[PDF]
|
|
|
|
|
|
|
P. M. Mannucci
Thrombotic thrombocytopenic purpura and the hemolytic uremic syndrome: much progress and many remaining issues
Haematologica,
July 1, 2007;
92(7):
878 - 880.
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. Ferrari, F. Scheiflinger, M. Rieger, G. Mudde, M. Wolf, P. Coppo, J.-P. Girma, E. Azoulay, C. Brun-Buisson, F. Fakhouri, et al.
Prognostic value of anti-ADAMTS13 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 ADAMTS13 activity
Blood,
April 1, 2007;
109(7):
2815 - 2822.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. N. George
Evaluation and Management of Patients With Thrombotic Thrombocytopenic Purpura
J Intensive Care Med,
March 1, 2007;
22(2):
82 - 91.
[Abstract]
[PDF]
|
|
|
|
|
|
|
P. M. Mannucci and F. Peyvandi
TTP and ADAMTS13: When Is Testing Appropriate?
Hematology,
January 1, 2007;
2007(1):
121 - 126.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
H. Izzedine, C. Isnard-Bagnis, V. Launay-Vacher, L. Mercadal, I. Tostivint, O. Rixe, I. Brocheriou, E. Bourry, S. Karie, S. Saeb, et al.
Gemcitabine-induced thrombotic microangiopathy: a systematic review
Nephrol. Dial. Transplant.,
November 1, 2006;
21(11):
3038 - 3045.
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. N. George
Clinical practice. Thrombotic thrombocytopenic purpura.
N. Engl. J. Med.,
May 4, 2006;
354(18):
1927 - 1935.
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. E. Sadler
Thrombotic Thrombocytopenic Purpura: A Moving Target
Hematology,
January 1, 2006;
2006(1):
415 - 420.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
