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目的:探讨利妥昔单抗治疗儿童难治性自身免疫性溶血性贫血(AIHA)的疗效、毒副作用。方法:(1)总结2例难治性AIHA患儿的临床表现、实验室检查结果及使用利妥昔单抗治疗前后的情况。(2)对2例患儿选用利妥昔单抗治疗(每次375 mg/m2,每周1次,共4次),随访利妥昔单抗使用后CD20+细胞、血红蛋白等的变化情况,探讨利妥昔单抗治疗儿童AIHA的疗效;同时观察发热、皮疹、感染等毒副作用。结果:2例患儿诊断AIHA后均首先选用糖皮质激素治疗,初期都能显效,但不能长期维持,需长期依赖激素及输血。使用利妥昔单抗治疗后,2例患儿均能显效,其中1例治愈,1例病情反复,最终死于严重贫血。结论:尽管糖皮质激素是治疗AIHA的一线药物,但激素依赖或难治性病例较常见,因此利妥昔单抗已逐渐成为儿童难治性AIHA安全有效的二线治疗药物。
Objective: To investigate the efficacy and side effects of rituximab in the treatment of children refractory autoimmune hemolytic anemia (AIHA). Methods: (1) To summarize the clinical manifestations, laboratory findings and the situation before and after treatment with rituximab in 2 refractory AIHA children. (2) Rituximab (375 mg / m2 every time, once a week for 4 times) was used in 2 patients. The change of CD20 + cells and hemoglobin after the use of rituximab was followed up, To investigate the efficacy of rituximab in the treatment of children with AIHA. At the same time, to observe the side effects of fever, rash and infection. Results: Two cases were diagnosed with AIHA after the first choice of glucocorticoid therapy, the initial can be effective, but not long-term maintenance, the need for long-term dependence on hormones and blood transfusions. After treatment with rituximab, two cases of children can be markedly effective, of which 1 case of cure, 1 case of repeated illness, and eventually died of severe anemia. Conclusion: Although glucocorticoids are first-line drugs for the treatment of AIHA, hormone-dependent or refractory cases are more common, so rituximab has gradually become a safe and effective second-line treatment for refractory AIHA in children.