Rituximab therapy for childhood Evans syndrome

被引:41
作者
Bader-Meunier, Brigitte [1 ]
Aladjidi, Nathalie [2 ]
Bellmann, Francoise [3 ]
Monpoux, Fabrice [4 ]
Nelken, Brigitte [5 ]
Robert, Alain [6 ]
Armari-Alla, Corinne [7 ]
Picard, Capucine [8 ]
Ledeist, Francoise [9 ]
Munzer, Martine [10 ]
Yacouben, Karima [1 ]
Bertrand, Yves [11 ]
Pariente, Antoine [12 ]
Chausse, Arnaud [2 ]
Perel, Yves [2 ]
Leverger, Guy [3 ]
机构
[1] Hop Robert Debre, AP HP, Serv Hematol Oncol, Paris, France
[2] Hop Pellegrin, Serv Hematol Oncol Pediat, Bordeaux, France
[3] Hop Armand Trousseau, AP HP, Serv Hematol Immunol Oncol Pediat, Paris, France
[4] Hop Archet II, Serv Hematol Immunol Oncol Rhumatol Pediat, Nice, France
[5] Hop Jeanne de Flandre, Serv Hematol Oncol Pediat, Lille, France
[6] Hop Enfants, Serv Hematol Oncol Pediat, Toulouse, France
[7] Hop La Tronche, Serv Hematol Oncol Pediat, La Tronche, France
[8] Hop Necker Enfants Malad, Hop Paris, AP HP, Ctr Etud Deficits Immunitaires, Paris, France
[9] Univ Montreal, Dept Microbiol & Immunol, CHU St Justine, Montreal, PQ, Canada
[10] Amer Mem Hosp, Unite Oncohematol Pediat, Reims, France
[11] Hop Debrousse, Unite Oncohematol Pediat, Lyon, France
[12] Univ Bordeaux 2, Dept Pharmacol, INSERM U657, Hop Pellegrin, Bordeaux, France
关键词
Evans syndrome; children; autoimmune hemolytic anemia; autoimmune thrombocytopenic purpura; rituximab;
D O I
10.3324/haematol.11540
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
The safety and efficacy of rituximab have been retrospectively assessed in 17 children with Evans syndrome. Patients received 4 or 3 weekly doses of rituximab (375 mg/m(2) per dose) associated with prednisone, alone (14 patients) or associated with other immunosuppressive drugs. Complete or partial remission of at least one cytopenia was achieved in 13 out of the 17 patients (76%), and lasted in 11 of them with a mean follow-up of 2.4 years (range 0.5-7 years). Steroid therapy was stopped or tapered at 50-100% of the baseline dosage in all long-term responders. Moderate side effects and infection occurred only in 4 and 1 children respectively.
引用
收藏
页码:1691 / 1694
页数:4
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