Dev Maha, Mushtaq Naureen, Faisal Ali
J Pak Med Assoc. 2014 Jun;64(6):700-2.
Autoimmune haemolytic anaemia is characterised by the formation of auto-antibodies that bind to the erythrocyte surface membrane, leading to haemolysis. It is the main cause of acquired extracorpuscular haemolysis in children. It can be classified according to the characteristic temperature reactivity of the red blood cell auto-antibody in warm-antibody autoimmune haemolytic anaemia (reacting at 37 degrees C) and cold-antibody autoimmune haemolytic anaemia (reacting optimally at lower temperature). Glucocorticoids and/or intravenous immunoglobulins are the mainstay of treatment in majority of patients with warm autoimmune haemolytic anaemia, but when these treatments fail, patients often require cytotoxic drugs or splenectomy. Rituximab, an anti-CD 20 monoclonal antibody, has gained widespread acceptance in the management of B-Cell malignancies. Additionally, it has been used to treat the disorders associated with auto-antibody production. We describe a 9-year-old boy with warm autoimmune haemolytic anaemia resistant to the standard treatment who was successfully treated with Rituximab.
自身免疫性溶血性贫血的特征是形成与红细胞表面膜结合的自身抗体，从而导致溶血。它是儿童获得性体外溶血的主要原因。根据红细胞自身抗体在温抗体自身免疫性溶血性贫血（在37℃反应）和冷抗体自身免疫性溶血性贫血（在较低温度下最佳反应）中的特征温度反应性，可对其进行分类。糖皮质激素和/或静脉注射免疫球蛋白是大多数温抗体自身免疫性溶血性贫血患者的主要治疗方法，但当这些治疗失败时，患者通常需要细胞毒性药物或脾切除术。利妥昔单抗，一种抗CD20单克隆抗体，在B细胞恶性肿瘤的治疗中已得到广泛认可。此外，它还被用于治疗与自身抗体产生相关的疾病。我们描述了一名9岁患有温抗体自身免疫性溶血性贫血且对标准治疗耐药的男孩，他通过利妥昔单抗成功治愈。
