Jaime-Pérez José Carlos, Aguilar-Calderón Patrizia, Salazar-Cavazos Lorena, Gómez-De León Andrés, Gómez-Almaguer David
Department of Hematology, Internal Medicine Division, Dr. Josè E. González University Hospital, School of Medicine of the Universidad Autónoma de Nuevo León, Monterrey, Mexico.
Blood Res. 2019 Jun;54(2):131-136. doi: 10.5045/br.2019.54.2.131. Epub 2019 Jun 25.
Warm autoimmune hemolytic anemia (w-AIHA) is an uncommon disease with heterogeneous response to treatment. Steroids are the standard treatment at diagnosis, whereas rituximab has recently been recommended as the second-line therapy of choice. Our main objective was to document the response to treatment in patients with newly diagnosed w-AIHA, including the effectiveness of low-dose rituximab as frontline treatment and for refractory disease.
Patients with w-AIHA from 2002 to 2017 were included. Relapse-free survival (RFS), probability of maintained response (MR), and time-to-response were analyzed using the Kaplan-Meier method. Response was classified as complete, partial, and no response.
We included 64 adults with w-AIHA (39 women and 25 men). The median age was 37 (16-77) years. Response rates to steroids alone were 76.7%, rituximab plus steroids, 100%; and cyclophosphamide, 80%. RFS with steroids at 6, 36, and 72 months was 86.3%, 65.1%, and 59.7%, respectively. Eighteen patients received rituximab at 100 mg/wk for 4 weeks plus high-dose dexamethasone as first-line therapy, with RFS at 6, 36, and 72 months of 92.3%, 58.7% and 44.1%, respectively. Eight patients refractory to several lines of therapy were treated with low-dose rituximab, and all achieved a response (three complete response and five partial response) at a median 16 days (95% confidence interval, 14.1-17.8), with a 75% probability of MR at 103 months; the mean MR was 81.93±18 months.
Outcomes of w-AIHA treatment were considerably heterogeneous. Low rituximab doses plus high dexamethasone doses were effective for refractory disease.
温抗体型自身免疫性溶血性贫血（w - AIHA）是一种罕见疾病，对治疗的反应具有异质性。类固醇是诊断时的标准治疗方法，而利妥昔单抗最近被推荐作为二线治疗的首选。我们的主要目标是记录新诊断的w - AIHA患者的治疗反应，包括低剂量利妥昔单抗作为一线治疗和用于难治性疾病的有效性。
纳入2002年至2017年的w - AIHA患者。使用Kaplan - Meier方法分析无复发生存期（RFS）、维持缓解概率（MR）和缓解时间。缓解分为完全缓解、部分缓解和无缓解。
我们纳入了64例成人w - AIHA患者（39名女性和25名男性）。中位年龄为37岁（16 - 77岁）。单独使用类固醇的缓解率为76.7%，利妥昔单抗加类固醇为100%；环磷酰胺为80%。使用类固醇在6个月、36个月和72个月时的RFS分别为86.3%、65.1%和59.7%。18例患者接受利妥昔单抗100mg/周，共4周加高剂量地塞米松作为一线治疗，6个月、36个月和72个月时的RFS分别为92.3%、58.7%和44.1%。8例对多线治疗难治的患者接受低剂量利妥昔单抗治疗，所有患者均在中位16天（95%置信区间，14.1 - 17.8）达到缓解（3例完全缓解和5例部分缓解），103个月时MR概率为75%；平均MR为81.93±18个月。
w - AIHA治疗的结果存在很大异质性。低剂量利妥昔单抗加高剂量地塞米松对难治性疾病有效。
