Makis Alexandros, Kanta Zoi, Kalogeropoulos Dimitrios, Chaliasos Nikoloaos
Department of Pediatrics, University Hospital of Ioannina, Ioannina, Greece.
Case Rep Hematol. 2018 Aug 26;2018:8471073. doi: 10.1155/2018/8471073. eCollection 2018.
Autoimmune hemolytic anemia (AIHA) is a relatively uncommon hematological entity in children and sometimes is characterized by a severe course requiring more than one line course therapy. Treatment decisions depend on the severity and chronicity of the anemia and the characteristics of the autoantibodies. Immunosuppression with corticosteroids is the first-line treatment, especially in warm-reactive AIHA. Refractory cases are treated with immunosuppressive drugs, cytotoxic agents, androgens, or splenectomy, with various side effects and questionable efficacy. Another second-line option is rituximab, an anti-CD20 monoclonal antibody, which has been used as an off-label agent with encouraging results from small limited studies or case reports. Herein, we add our experience on the safety and clinical efficacy of rituximab by presenting the case of a boy with warm-type AIHA resistant to corticosteroids and azathioprine, successfully treated with rituximab. We also offer a review of the relevant literature.
自身免疫性溶血性贫血（AIHA）在儿童中是一种相对罕见的血液学疾病，有时其病程严重，需要多种治疗方案。治疗决策取决于贫血的严重程度和慢性程度以及自身抗体的特征。使用糖皮质激素进行免疫抑制是一线治疗方法，尤其是在温反应性AIHA中。难治性病例采用免疫抑制药物、细胞毒性药物、雄激素或脾切除术治疗，这些治疗方法有各种副作用且疗效存疑。另一种二线选择是利妥昔单抗，一种抗CD20单克隆抗体，已作为一种标签外药物使用，小型有限研究或病例报告显示出令人鼓舞的结果。在此，我们通过介绍一名对糖皮质激素和硫唑嘌呤耐药的温型AIHA男孩成功接受利妥昔单抗治疗的病例，分享我们关于利妥昔单抗安全性和临床疗效的经验。我们还对相关文献进行了综述。
