Efficacy and safety of rituximab in common variable immunodeficiency-associated immune cytopenias: a retrospective multicentre study on 33 patients

被引：99

作者：

Gobert, Delphine ^{[1
]}

Bussel, James B. ^{[2
,3
]}

Cunningham-Rundles, Charlotte ^{[4
]}

Galicier, Lionel ^{[5
]}

Dechartres, Agnes ^{[6
]}

Berezne, Alice ^{[7
]}

Bonnotte, Bernard ^{[8
]}

DeRevel, Thierry ^{[9
]}

Auzary, Christophe ^{[10
]}

Jaussaud, Roland ^{[11
]}

Larroche, Claire ^{[12
]}

LeQuellec, Alain ^{[13
]}

Ruivard, Marc ^{[14
]}

Seve, Pascal ^{[15
]}

Smail, Amar ^{[16
]}

Viallard, Jean-Francois ^{[17
]}

Godeau, Bertrand ^{[1
]}

Hermine, Olivier ^{[18
]}

Michel, Marc ^{[1
]}

机构：

[1] Hop Henri Mondor, Dept Internal Med, Paris, France

[2] NY Presbyterian Hosp, Div Pediat Hematol, New York, NY USA

[3] Weill Cornell Med Coll, New York, NY USA

[4] Mt Sinai Sch Med, New York, NY USA

[5] Hop St Louis, Dept Immunohaematol, Paris, France

[6] Univ Paris 05, Hop Hotel Dieu, APHP, Ctr Clin Epidemiol,INSERM U 738, Sorbonne Paris Cite, France

[7] Hop Cochin, Dept Internal Med, F-75674 Paris, France

[8] Hop Le Bocage, Dept Internal Med, Dijon, France

[9] Hop Percy, Dept Haematol, Clamart, France

[10] Hop Robert Bisson, Dept Internal Med, Lisieux, France

[11] Hop Robert Debre, Dept Internal Med, Reims, France

[12] Hop Avicenne, Dept Internal Med, F-93009 Bobigny, France

[13] Hop St Eloi, Dept Internal Med, Montpellier, France

[14] Ctr Hosp Univ, Dept Internal Med, Clermont Ferrand, France

[15] Hop Hotel Dieu, Dept Internal Med, F-69288 Lyon, France

[16] Hop Nord Amiens, Dept Internal Med, Amiens, France

[17] Hop Haut Leveque, Dept Internal Med, Pessac, France

[18] Hop Necker Enfants Malad, Dept Haematol, Paris, France

来源：

BRITISH JOURNAL OF HAEMATOLOGY | 2011年 / 155卷 / 04期

关键词：

immune thrombocytopenia; autoimmune haemolytic anaemia; Evans' syndrome; common variable immunodeficiency; rituximab; ANTI-CD20; MONOCLONAL-ANTIBODY; AUTOIMMUNE HEMOLYTIC-ANEMIA; THROMBOCYTOPENIC PURPURA; ADULTS; DEPLETION; CRITERIA; DISEASES; CHILDREN; THERAPY;

D O I：

10.1111/j.1365-2141.2011.08880.x

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Patients with common variable immunodeficiency (CVID) are at high risk of developing immune thrombocytopenia (ITP) and/or autoimmune haemolytic anaemia (AHA). Given their underlying immunodeficiency, immunosuppressive treatment of these manifestations may increase the risk of infection. To assess efficacy and safety of rituximab in patients with CVID-associated ITP/AHA, a multicentre retrospective study was performed. Thirty-three patients, 29 adults and four children, were included. Patients received an average of 2 6 treatments prior to rituximab including steroids, intravenous immunoglobulin and splenectomy (21%). The median ITP/AHA duration at time of first rituximab administration was 12 months [range 1-324] and the indication for using rituximab was ITP (22 cases), AHA (n = 5) or both (n = 7); 1 patient was treated sequentially for ITP and then AHA. The overall initial response rate to rituximab was 85% including 74% complete responses. After a mean follow-up of 39 +/- 30 months after rituximab first administration, 10 of the initial responders relapsed and re-treatment with rituximab was successful in 7/9. Severe infections occurred after rituximab in eight adults (24%), four of whom were not on immunoglobulin replacement therapy. In conclusion, rituximab appears to be highly effective and relatively safe for the management of CVID-associated severe immune cytopenias.

引用

页码：498 / 508

页数：11

共 32 条

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