Awasthi Shankar, Upreti Sunita
Department of Internal Medicine, Greater Baltimore Medical Center, Towson, Maryland, USA.
J Community Hosp Intern Med Perspect. 2020 Sep 3;10(5):470-474. doi: 10.1080/20009666.2020.1787811.
Macrophage Activation Syndrome (MAS) is a potentially fatal inflammatory condition that can rapidly lead to multi-organ failure if inadequately treated. Also, known as secondary Hemophagocytic Lymphohistiocytosis (sHLH), MAS is commonly seen as a complication of systemic inflammatory disorders, like systemic juvenile idiopathic arthritis (sJIA) and systemic lupus erythematosus (SLE). However, MAS can also present as a complication of malignancies and infections, particularly viral infections like Epstein-Barr virus (EBV), cytomegalovirus (CMV) and HIV. Here we describe a patient with an underlying history of SLE and Sjogren's disease who was found have both EBV and CMV infections, presented to our facility with fever, lymphadenopathy, pneumonia and pancytopenia. Patient was treated in line with sepsis in the intensive care unit but rapidly developed multigrain failure despite early aggressive treatment. As will be discussed below, patient had characteristic signs and symptoms of MAS with biochemical parameters pointing towards the same.
巨噬细胞活化综合征（MAS）是一种潜在的致命性炎症性疾病，如果治疗不当，可迅速导致多器官功能衰竭。MAS也被称为继发性噬血细胞性淋巴组织细胞增生症（sHLH），通常被视为系统性炎症性疾病的并发症，如系统性幼年特发性关节炎（sJIA）和系统性红斑狼疮（SLE）。然而，MAS也可能表现为恶性肿瘤和感染的并发症，特别是像爱泼斯坦-巴尔病毒（EBV）、巨细胞病毒（CMV）和人类免疫缺陷病毒（HIV）等病毒感染。在此，我们描述一名患有SLE和干燥综合征病史的患者，该患者被发现同时感染了EBV和CMV，因发热、淋巴结病、肺炎和全血细胞减少症前来我院就诊。患者在重症监护病房按照脓毒症进行治疗，但尽管早期积极治疗，仍迅速出现多器官功能衰竭。如下文将讨论的，患者具有MAS的典型体征和症状，生化指标也指向这一点。
