Division of Pediatric Hematology Oncology, Childrens Hospital Los Angeles, Los Angeles, California 90027, USA.
Pediatr Blood Cancer. 2011 Mar;56(3):460-2. doi: 10.1002/pbc.22830. Epub 2010 Nov 11.
Hemophagocytic lymphohistiocytosis (HLH) is characterized by fever, cytopenias, splenomegaly, and hemophagocytosis by macrophages activated by high cytokine levels. Chronic granulomatous disease (CGD) is characterized by recurrent infections, hyperinflammation, and excessive cytokine release. This may predispose patients with CGD to developing HLH during an infection. We conducted a retrospective review of patients with CGD, treated at our institution between 1999 and 2008. Three out of 17 patients developed HLH. Patients with CGD may be at increased risk for developing HLH. Remission of HLH was achieved after treatment with antimicrobials, steroids, and intravenous immunoglobulin This approach to treatment appears to be effective.
噬血细胞性淋巴组织细胞增生症（HLH）的特征为发热、血细胞减少、脾大以及被高细胞因子水平激活的巨噬细胞吞噬血细胞。慢性肉芽肿病（CGD）的特征为反复感染、高炎症反应和过度细胞因子释放。这可能使 CGD 患者在感染期间易发生 HLH。我们对 1999 年至 2008 年在我院接受治疗的 CGD 患者进行了回顾性研究。17 例患者中有 3 例发生 HLH。CGD 患者发生 HLH 的风险可能增加。在使用抗生素、类固醇和静脉注射免疫球蛋白治疗后，HLH 缓解。这种治疗方法似乎有效。
