APHP, Henri Mondor Hospital, Department of Internal Medicine, French National Referral Center For Adults 'Immune Cytopenias, Créteil, France.
Am J Hematol. 2014 Sep;89(9):E150-5. doi: 10.1002/ajh.23767. Epub 2014 Jun 19.
Warm autoimmune hemolytic anemia (wAIHA) is a rare autoimmune disease with poorly known natural history and management remaining mainly empirical. To better describe the characteristics and outcome of wAIHA in adults, we performed a single-center cohort study of patients diagnosed with wAIIHA from 2001 to 2012 in our center. Sixty patients (50% women) were included, the mean age at the time of wAIHA onset was 54 ± 23 years. wAIHA was considered "primary" for 21 patients (35%) and was associated with an underlying disorder in 39 (65%), including mainly lymphoproliferative disorders and systemic lupus. All patients but two needed treatment and received corticosteroids, with an overall initial response rate of 87%. However, 63% of the patients were corticosteroid-dependent and 56% required at least one second-line treatment including mainly rituximab (n = 19). At the time of analysis, after a mean follow-up of 46 months, 28 patients (47%) were in remission and off treatment and 5 (8%) had died. The presence of an underlying lymphoproliferative disorder was associated with reduced response to corticosteroids and increased need for second-line therapy. In conclusion, in the last decade and compared to a previous series from our center, the rate of secondary wAIHA has increased and the use of rituximab has emerged as the preferred second-line treatment and corticosteroid-sparing strategy; the overall mortality has significantly decreased (8 vs. 18%).
温抗体型自身免疫性溶血性贫血（wAIHA）是一种罕见的自身免疫性疾病，其自然病史和治疗方法知之甚少，主要依赖经验。为了更好地描述成人 wAIHA 的特征和结局，我们对 2001 年至 2012 年在我院诊断为 wAIIHA 的患者进行了一项单中心队列研究。共纳入 60 例患者（50%为女性），wAIHA 发病时的平均年龄为 54±23 岁。21 例（35%）患者被认为是“原发性”，39 例（65%）患者与潜在疾病相关，主要包括淋巴增生性疾病和系统性红斑狼疮。所有患者均需要治疗，接受了皮质类固醇治疗，总体初始缓解率为 87%。然而，63%的患者依赖于皮质类固醇，56%的患者需要至少一种二线治疗，主要包括利妥昔单抗（n=19）。在分析时，经过平均 46 个月的随访，28 例（47%）患者缓解并停止治疗，5 例（8%）患者死亡。潜在的淋巴增生性疾病与皮质类固醇反应降低和二线治疗需求增加相关。总之，与我院之前的系列研究相比，在过去十年中，继发性 wAIHA 的发生率增加，利妥昔单抗的应用已成为首选的二线治疗和皮质类固醇节约策略；总体死亡率显著下降（8 例 vs. 18%）。
