Camou F, Viallard J-F, Pellegrin J-L
Service de médecine interne, centre François-Magendie, hôpital du Haut-Lévêque, CHU de Bordeaux, avenue Magellan, 33604 Pessac cedex, France.
Rev Med Interne. 2003 Aug;24(8):501-4. doi: 10.1016/s0248-8663(03)00139-5.
Cold agglutinin disease is a chronic auto-immune hemolytic anemia related to a lymphoproliferative disorder with a degenerative potential and no codified treatment. This rare affection is related to the production of anti-erythrocytes immunoglobulins M. They are responsible of hemolytic crises sometimes severe and vascular acrosyndrom when submitted to cold temperature. Before rituximab, a monoclonal antibody targeted against the B lymphocyte CD20 antigen, no treatment was really efficient.
We present 5 patients who have been treated with 4 weekly rituximab perfusions, and then we proceed to a review of the literature concerning the other 23 similar cases.
With a good tolerance, the treatment allowed a remission in all the cases (4 partial, 1 complete). Among the 23 observations published, the rate of answer was 21/23 (of which 14 gave completes).
Rituximab is an alternative treatment of cold agglutinin disease.
冷凝集素病是一种慢性自身免疫性溶血性贫血,与具有退行性潜能且无规范治疗方法的淋巴增殖性疾病相关。这种罕见病症与抗红细胞免疫球蛋白M的产生有关。当暴露于低温时,它们会引发有时严重的溶血危机和血管性紫癜。在利妥昔单抗(一种靶向B淋巴细胞CD20抗原的单克隆抗体)出现之前,没有真正有效的治疗方法。
我们介绍了5例接受4周利妥昔单抗灌注治疗的患者,然后对其他23例类似病例的文献进行了综述。
该治疗耐受性良好,所有病例均实现缓解(4例部分缓解,1例完全缓解)。在已发表的23例观察病例中,有效率为21/23(其中14例完全缓解)。
利妥昔单抗是冷凝集素病的一种替代治疗方法。