Benefit of Anakinra in Treating Pediatric Secondary Hemophagocytic Lymphohistiocytosis
Benefit of Anakinra in Treating Pediatric Secondary Hemophagocytic Lymphohistiocytosis
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DOI：
10.1002/art.41103
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发表时间：
2019-12-26
影响因子：
13.3
通讯作者：
Cron, Randy Q.
中科院分区：
文献类型：
--
作者：
Eloseily, Esraa M.;Weiser, Peter;Cron, Randy Q.
目的 评估重组人白细胞介素 - 1受体拮抗剂阿那白滞素在治疗风湿性和非风湿性疾病相关的继发性噬血细胞性淋巴组织细胞增生症（HLH）/巨噬细胞活化综合征（MAS）儿科患者中的益处。方法 对2008年1月至2016年12月在阿拉巴马儿童医院所有接受阿那白滞素治疗的继发性HLH/MAS患者进行回顾性病历审查。从病历中收集人口统计学、临床、实验室和遗传学特征、结果数据以及同时进行的治疗信息，并使用适当的单变量统计方法进行分析，以评估治疗后的变化以及患者变量与结果之间的关联。结果 在电子病历中确定了44例接受阿那白滞素治疗的继发性HLH/MAS患者。住院中位时间为15天。治疗前平均血清铁蛋白水平为33316 ng/ml，在阿那白滞素治疗开始后的15天内降至14435 ng/ml（下降57%）。该队列的总体死亡率为27%。较早开始使用阿那白滞素（住院5天内）与死亡率降低相关（P = 0.046），而血小板减少症（血小板计数……
Objective To assess the benefit of the recombinant human interleukin-1 receptor antagonist anakinra in treating pediatric patients with secondary hemophagocytic lymphohistiocytosis (HLH)/macrophage activation syndrome (MAS) associated with rheumatic and nonrheumatic conditions. Methods A retrospective chart review of all anakinra-treated patients with secondary HLH/MAS was performed at Children's of Alabama from January 2008 through December 2016. Demographic, clinical, laboratory, and genetic characteristics, outcomes data, and information on concurrent treatments were collected from the records and analyzed using appropriate univariate statistical approaches to assess changes following treatment and associations between patient variables and outcomes. Results Forty-four patients with secondary HLH/MAS being treated with anakinra were identified in the electronic medical records. The median duration of hospitalization was 15 days. The mean pretreatment serum ferritin level was 33,316 ng/ml and dropped to 14,435 ng/ml (57% decrease) within 15 days of the start of anakinra treatment. The overall mortality rate in the cohort was 27%. Earlier initiation of anakinra (within 5 days of hospitalization) was associated with reduced mortality (P = 0.046), whereas thrombocytopenia (platelet count