Argüello Marina María, López Rubio Montserrat, Castilla García Lucía
Servicio de Hematología y Hemoterapia, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Madrid, España.
Servicio de Hematología y Hemoterapia, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Madrid, España.
Med Clin (Barc). 2023 Jan 5;160(1):30-38. doi: 10.1016/j.medcli.2022.07.021. Epub 2022 Nov 2.
Autoimmune haemolytic anaemias (AIHA) are acquired haematological disorders caused by increased peripheral erythrocyte destruction mediated by autoantibodies against erythrocyte antigens. They classified according to aetiology into primary and secondary, and according to the type of antibody and reaction temperature into AIHA due to warm antibodies (w-AIHA) and AIHA due to cold antibodies (c-AIHA). The mainstay of management in w-AIHA remains glucocorticoid therapy, and the early addition of rituximab has shown good results in recent studies. Primary c-AIHA is mainly treated with rituximab, alone or in combination with chemotherapy. New drugs such as Syk inhibitors, anti-FcRn Ig and complement inhibitors are in advanced development and will expand the therapeutic arsenal, especially in refractory or relapsed cases.
自身免疫性溶血性贫血（AIHA）是一种获得性血液系统疾病，由针对红细胞抗原的自身抗体介导的外周红细胞破坏增加所致。根据病因，它们可分为原发性和继发性；根据抗体类型和反应温度，可分为温抗体型自身免疫性溶血性贫血（w-AIHA）和冷抗体型自身免疫性溶血性贫血（c-AIHA）。w-AIHA治疗的主要手段仍然是糖皮质激素疗法，最近的研究表明，早期加用利妥昔单抗效果良好。原发性c-AIHA主要用利妥昔单抗单独或联合化疗进行治疗。脾酪氨酸激酶（Syk）抑制剂、抗新生儿同种免疫性血小板减少症（FcRn）免疫球蛋白和补体抑制剂等新药正处于研发后期，将扩大治疗手段，特别是在难治性或复发性病例中。
