Shanafelt Tait D, Madueme Hans L, Wolf Robert C, Tefferi Ayalew
Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, Minn 55905, USA.
Mayo Clin Proc. 2003 Nov;78(11):1340-6. doi: 10.4065/78.11.1340.
To evaluate the efficacy of rituximab for the treatment of adult patients with immune cytopenia, including idiopathic thrombocytopenic purpura (ITP), autoimmune hemolytic anemia, and Evans syndrome.
We retrospectively reviewed the medical charts of all patients treated with rituximab for immune cytopenia at the Mayo Clinic in Rochester, Minn, through January 1, 2003. Fourteen patients (median age at first diagnosis, 51 years; range, 21-79 years) were identified who received 1 or more treatment courses of rituximab for treatment of refractory ITP (12 patients), autoimmune hemolytic anemia (AIHA) (5 patients), or both ITP and AIHA (classified as Evans syndrome) (4 patients). Data regarding age, diagnosis, date of diagnosis, previous treatments, comorbid conditions, blood cell counts before taking rituximab, number of rituximab treatments, and response to treatment were extracted and analyzed.
Of 12 patients treated for ITP, 6 were receiving corticosteroid-based treatment either alone or combined with other immunosuppressive therapy at the time they received rituximab. Complete remission occurred in 5 (42%) of 12 patients with ITP and in 2 (40%) of 5 patients with AIHA. Response to rituximab in patients with Evans syndrome was seen in either ITP or AIHA, but not both. Complete response was often durable in ITP. Responses were seen in both splenectomized and nonsplenectomized patients.
Our findings, considered with the results of other studies, suggest that rituximab deserves early consideration as salvage therapy for immune cytopenias that are refractory to both corticosteroid treatment and splenectomy. This series represents the largest series of adult patients with AIHA and Evans syndrome.
评估利妥昔单抗治疗成年免疫性血细胞减少症患者的疗效,这些患者包括特发性血小板减少性紫癜(ITP)、自身免疫性溶血性贫血和伊文氏综合征。
我们回顾性分析了明尼苏达州罗切斯特市梅奥诊所截至2003年1月1日接受利妥昔单抗治疗免疫性血细胞减少症的所有患者的病历。确定了14例患者(首次诊断时的中位年龄为51岁;范围为21 - 79岁),他们接受了1个或更多疗程的利妥昔单抗治疗,用于治疗难治性ITP(12例患者)、自身免疫性溶血性贫血(AIHA)(5例患者)或ITP和AIHA两者(归类为伊文氏综合征)(4例患者)。提取并分析了有关年龄、诊断、诊断日期、既往治疗、合并症、服用利妥昔单抗前的血细胞计数、利妥昔单抗治疗次数以及治疗反应的数据。
在接受ITP治疗的12例患者中,6例在接受利妥昔单抗治疗时单独或联合其他免疫抑制疗法接受基于皮质类固醇的治疗。12例ITP患者中有5例(42%)实现完全缓解,5例AIHA患者中有2例(40%)实现完全缓解。伊文氏综合征患者对利妥昔单抗的反应在ITP或AIHA中出现,但并非两者都出现。ITP患者的完全缓解通常具有持久性。脾切除和未脾切除的患者均出现反应。
我们的研究结果与其他研究结果一起表明,对于对皮质类固醇治疗和脾切除均难治的免疫性血细胞减少症,利妥昔单抗作为挽救疗法值得早期考虑。本系列代表了最大的成年AIHA和伊文氏综合征患者系列。