Dumancas Carissa Y, Reyes Hans Alexi Garay, Cosico Juan, Savadkar Amrut, Lah Soowhan
Department of Medicine, New York Medical College, Metropolitan Hospital Center, New York, NY, USA.
Am J Case Rep. 2018 Jan 8;19:25-28. doi: 10.12659/ajcr.906590.
BACKGROUND Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening condition that has a poor prognosis due to the ensuing cytokine storm leading to severe organ damage. Current treatment guidelines suggest using a combination of steroid- and etoposide-based chemotherapy. CASE REPORT The authors present a case of a 41-year-old African-American female who presented with symptoms of foodborne illness and who developed multi-organ dysfunction. HLH was suspected because of poor response to broad-spectrum antibiotics with a constellation of findings, including cytopenia, hypofibrinogenemia, hypertriglyceridemia, and hyperferritinemia. Clinical improvement was noted after administration of intravenous immunoglobulin and dexamethasone while waiting for the soluble interleukin-2 receptor levels; therefore, chemotherapy was not administered. CONCLUSIONS Despite the variable and poor prognosis of HLH, early treatment with steroids and immunosuppressive therapy is crucial to improving the survival rate. The inclusion of immunoglobulin therapy should be considered a treatment option for HLH.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见的危及生命的疾病，由于随之而来的细胞因子风暴导致严重器官损伤，其预后较差。目前的治疗指南建议使用基于类固醇和依托泊苷的联合化疗。病例报告：作者介绍了一例41岁的非裔美国女性病例，该患者出现食源性疾病症状并发展为多器官功能障碍。由于对广谱抗生素反应不佳且出现一系列症状，包括血细胞减少、低纤维蛋白原血症、高甘油三酯血症和高铁蛋白血症，怀疑为HLH。在等待可溶性白细胞介素-2受体水平结果期间，静脉注射免疫球蛋白和地塞米松后临床症状有所改善；因此未进行化疗。结论：尽管HLH的预后多变且较差，但早期使用类固醇和免疫抑制治疗对于提高生存率至关重要。免疫球蛋白治疗应被视为HLH的一种治疗选择。
