Autoimmune hemolytic anemias (AIHAs) are rare and heterogeneous disorders characterized by the destruction of red blood cells through warm or cold antibodies. There is currently no licensed treatment for AIHA. Due to the paucity of clinical trials, recommendations on diagnosis and therapy have often been based on expert opinions and some national guidelines. Here we report the recommendations of the First International Consensus Group, who met with the aim to review currently available data and to provide standardized diagnostic criteria and therapeutic approaches as well as an overview of novel therapies. Exact diagnostic workup is important because symptoms, course of disease, and therapeutic management relate to the type of antibody involved. Monospecific direct antiglobulin test is considered mandatory in the diagnostic workup, and any causes of secondary AIHA have to be diagnosed. Corticosteroids remain first-line therapy for warm-AIHA, while the addition of rituximab should be considered early in severe cases and if no prompt response to steroids is achieved. Rituximab with or without bendamustine should be used in the first line for patients with cold agglutinin disease requiring therapy. We identified a need to establish an international AIHA network. Future recommendations should be based on prospective clinical trials whenever possible.

自身免疫性溶血性贫血（AIHA）是罕见的异质性疾病，其特征是通过热抗体或冷抗体破坏红细胞。当前没有AIHA的许可治疗。由于缺乏临床试验，有关诊断和治疗的建议通常基于专家意见和一些国家指南。在这里，我们报告第一国际共识小组的建议，该小组的目的是审查当前可用的数据，并提供标准化的诊断标准和治疗方法以及新疗法的概述。准确的诊断检查非常重要，因为症状，病程和治疗管理与所涉及抗体的类型有关。在诊断检查中，单特异性直接抗球蛋白测试被认为是强制性的，并且必须诊断出继发性AIHA的任何原因。皮质类固醇仍是温暖AIHA的一线治疗，而在严重病例中，如果对类固醇没有迅速反应，应考虑加用利妥昔单抗。对于需要治疗的冷凝集素疾病患者，一线应使用利妥昔单抗加或不加苯达莫司汀。我们确定有必要建立国际AIHA网络。未来的建议应尽可能基于前瞻性临床试验。对于需要治疗的冷凝集素疾病的患者，一线应使用含或不含苯达莫司汀的利妥昔单抗。我们确定有必要建立国际AIHA网络。未来的建议应尽可能基于前瞻性临床试验。对于需要治疗的冷凝集素疾病的患者，一线应使用含或不含苯达莫司汀的利妥昔单抗。我们确定有必要建立国际AIHA网络。未来的建议应尽可能基于前瞻性临床试验。