免疫抑制治疗儿童重型再生障碍性贫血54例疗效分析  

Immunosuppressive therapy for 54 children patients with acquired severe aplastic anemia

作　　者：陈纯[1] 方建培[1] 黄绍良[1] 钟凤仪[1] 

机构地区：[1]中山大学附属第二医院儿科,广州510120

出　　处：《中华儿科杂志》2006年第11期841-844,共4页Chinese Journal of Pediatrics

摘　　要：目的探讨免疫抑制治疗儿童重型再生障碍性贫血(severe aplasia anemia,SAA)的疗效。方法回顾性分析我院1997年1月—2003年6月儿童重型再生障碍性贫血54例,应用环孢素A(cyclosporine,CSA)和抗胸腺细胞球蛋白(antithymic globlin,ATG)为主的免疫抑制治疗,其中应用CSA联合ATG治疗31例(称为CSA联合ATG组),应用CSA治疗23例(称为CSA组),比较两组的治疗有效率、复发率、不良反应和无病生存率。结果两组的分型和极重型患者资料具有可比性。CSA联合ATG组和CSA组起效时间平均分别为2.5个月和3.5个月,两组有效率分别为81%(25/31)和52%(12/23)(χ2=4.962,P<0.05)。治疗有效者共37例,CSA联合ATG组和CSA组的复发率分别为8%(2/25)和50%(6/12)(χC2=6.143,P<0.05)。两组患者不良反应的发生率差异无统计学意义。所有患者随访至少1年,CSA联合ATG组和CSA组1年以上生存率分别为81%(25/31)和52%(12/23);随访超过2年者共47例,CSA联合ATG组和CSA组2年以上生存率分别为74%(20/27)和50%(10/20)(P<0.01)。结论免疫抑制治疗儿童重型再生障碍性贫血的疗效肯定,而CSA联合ATG治疗重型再生障碍性贫血的疗效更优于单用CSA。Objective Aplastic anemia is characterized by bone marrow failure and marked reduction of white blood cells, red blood cells and platelets in peripheral blood. Clinical studies have shown that immunosuppressive therapy greatly prolonged the long-term survival of some patients with aplastie anemia. But in severe aplastie anemia （SAA） patients whose ANC was 〈 0. 5 × 10^9/L, platelets were 〈 20 × 10^9/L, very low bone marrow proliferation and high death rate were observed. The present study aimed to evaluate the efficacy of immunosuppressive treatments with eyelosporine A （CSA） alone or CSA combined with antithymoeyte globin （ATG） in children with acquired SAA. Methods Fifty-four cases with SAA were treated with immunosuppressive agents mentioned above in our department from Jan. 1997 to June 2003, 31 of the cases had treated with CSA combined with ATG. There were 18 cases with SAA type Ⅰ and 13 cases with SAA type Ⅱ in CSA combined with ATG group, and 13 cases had very severe aplastie anemia. The other 23 cases were treated with CSA alone （ CSA group） , 10 of these cases had SAA- Ⅰ and 13 had SAA- Ⅱ , and 5 cases had very severe aplastic anemia. The responsive rate, relapse, adverse reactions and event free survival （EFS） were compared between CSA combined with ATG group and CSA group. Re.suits The proportions of patients with different types of the disease and severity were comparable between the two groups. The responsive time of the CSA combined with ATG group and CSA group was 2. 5 months and 3.5 months, respectively （ P 〈 0. 05 ）, the responsive rate in two groups was 81% （ 25/31 ） and 52 % （ 12/23 ）, respoctively（x^2 =4. 962, P 〈0. 05）. In 37 cases who were responsive to therapy, the relapse rate was 8% （2/25） and 50% （ 6/12 ） respectively （xc^2 = 6. 143, P 〈 2 0. 05 ）. There were no significant differences in adverse reactions to the immunosuppressive agents. All cases were followed-up for more than 1 year, and the event-free survival over

关 键 词：贫血 再生障碍性 环孢菌素 抗淋巴细胞血清 

分 类 号：R725.5[医药卫生—儿科]