Kamel Ralph, Sharma Rubal, Asti Divya, Gill Arshpal, Skaradinskiy Yevgeniy
Staten Island University Hospital, Northwell Health, 475 Seaview Avenue, Staten Island, New York 10305, NY, USA.
Case Rep Hematol. 2020 Dec 8;2020:8833196. doi: 10.1155/2020/8833196. eCollection 2020.
Hemophagocytic lymphohistiocytosis is a serious and potentially fatal disorder characterized by excessive immune system activation. The disorder is diagnosed mainly based on laboratory, clinical, and pathologic criteria. The spectrum comprises hereditary or "primary" HLH that comprises genetically heterogeneous conditions, occurring during childhood. The secondary form presents later in life and is associated with several conditions mainly malignancy, autoimmune diseases, viral or bacterial infections, and hematological diseases. We present the case of an 80-year-old female patient who initially presented with an acute viral syndrome secondary to respiratory syncytial virus. The hospital course was complicated by disseminated intravascular coagulation and shock with multiorgan failure. Extensive workup revealed that several of the criteria for hemophagocytic lymphohistiocytosis were met. A review of literature fails to identify cases of hemophagocytic lymphohistiocytosis associated with respiratory syncytial virus in immunocompetent adults. This case report provides further insight on RSV as a possible etiologic agent associated with HLH and the importance of early recognition of this fatal disorder in RSV-positive patients who show unpredictable clinical decompensation.
噬血细胞性淋巴组织细胞增生症是一种严重且可能致命的疾病，其特征为免疫系统过度激活。该疾病主要依据实验室、临床和病理标准进行诊断。其谱系包括遗传性或“原发性”HLH，这是一种基因异质性疾病，多在儿童期发病。继发性形式则在生命后期出现，与多种疾病相关，主要包括恶性肿瘤、自身免疫性疾病、病毒或细菌感染以及血液系统疾病。我们报告了一例80岁女性患者，最初表现为继发于呼吸道合胞病毒的急性病毒综合征。住院期间病情因弥散性血管内凝血和伴有多器官功能衰竭的休克而复杂化。全面检查发现符合噬血细胞性淋巴组织细胞增生症的多项标准。文献回顾未发现免疫功能正常的成年人中与呼吸道合胞病毒相关的噬血细胞性淋巴组织细胞增生症病例。本病例报告进一步揭示了呼吸道合胞病毒作为与HLH相关的可能病因，以及在出现不可预测临床失代偿的呼吸道合胞病毒阳性患者中早期识别这种致命疾病的重要性。
