D'Arena Giovanni, Califano Catello, Annunziata Mario, Tartarone Alfredo, Capalbo Silvana, Villani Oreste, Amendola Giovanni, Pietrantuono Giuseppe, Ferrara Felicetto, Pinto Antonio, Musto Pellegrino, D'Arco Alfonso Maria, Cascavilla Nicola
Hematology, Oncology and Bone Marrow Transplantation Unit, National Cancer Institute, Fondazione 'Pascale', Naples, Italy.
Eur J Haematol. 2007 Jul;79(1):53-8. doi: 10.1111/j.1600-0609.2007.00861.x. Epub 2007 May 28.
Warm-type idiopathic autoimmune hemolytic anemia (AIHA) is a relatively common hematologic disorder resulting from autoantibody production against red blood cells. Steroids represent the first-line therapeutic option, and immunosuppressive agents as well as splenectomy are used for refractory cases. Recently, the anti-CD20 monoclonal antibody rituximab has been shown to control autoimmune hemolysis in patients with refractory chronic disease. We report results from a retrospective analysis of 11 adult patients receiving rituximab for steroid-refractory AIHA of the warm type at a mean age of 55 yr (range 23-81 yr). All patients were given methyl-prednisolone as first-line treatment and some of them also received azathioprine and intravenous high-dose immunoglobulins. One patient underwent splenectomy. All patients were considered refractory to steroids and/or immunosuppressive drugs and all were then given weekly rituximab (375 mg/m(2)) for four consecutive weeks. An increase in hemoglobin (Hgb) levels in response to rituximab, with a mean increment of 3.3 g/dL (95% CI 2.1-4.4), was observed in all cases. Four patients required packed red cell transfusions before starting rituximab and all became transfusion-free. At a mean follow-up of 604 d (range 30-2884 d) since the treatment of AIHA with rituximab, all patients are alive, eight (73%) of them in complete remission (CR) and three (27%) in partial remission (PR). A moderate hemolysis still persisted in six (54%) patients. In conclusion, our experience clearly demonstrates that anti-CD20 monoclonal antibody rituximab is an effective and safe alternative treatment option for idiopathic AIHA, in particular, for steroid-refractory disease.
温抗体型自身免疫性溶血性贫血(AIHA)是一种相对常见的血液系统疾病,由针对红细胞的自身抗体产生所致。类固醇是一线治疗选择,免疫抑制剂以及脾切除术用于难治性病例。最近,抗CD20单克隆抗体利妥昔单抗已被证明可控制难治性慢性病患者的自身免疫性溶血。我们报告了对11例成年患者进行回顾性分析的结果,这些患者因温抗体型类固醇难治性AIHA接受利妥昔单抗治疗,平均年龄为55岁(范围23 - 81岁)。所有患者均接受甲泼尼龙作为一线治疗,其中一些患者还接受了硫唑嘌呤和静脉注射大剂量免疫球蛋白。1例患者接受了脾切除术。所有患者均被认为对类固醇和/或免疫抑制药物难治,随后均接受每周一次的利妥昔单抗(375 mg/m²),连续四周。所有病例均观察到血红蛋白(Hgb)水平因利妥昔单抗而升高,平均增加3.3 g/dL(95%可信区间2.1 - 4.4)。4例患者在开始使用利妥昔单抗前需要输注浓缩红细胞,而所有患者后来都不再需要输血。自使用利妥昔单抗治疗AIHA以来,平均随访604天(范围30 - 2884天),所有患者均存活,其中8例(73%)完全缓解(CR),3例(27%)部分缓解(PR)。6例(54%)患者仍存在中度溶血。总之,我们的经验清楚地表明,抗CD20单克隆抗体利妥昔单抗是特发性AIHA,特别是类固醇难治性疾病的一种有效且安全的替代治疗选择。