Kumral Abigail V W, Petersen William C, Heitz Christopher, Waggoner-Fountain Linda A, Belyea Brian C
*Department of Pediatrics †Division of Pediatric Hematology-Oncology ‡Department of Pathology §Division of Pediatric Infectious Diseases, University of Virginia, Charlottesville, VA.
J Pediatr Hematol Oncol. 2017 Aug;39(6):e325-e327. doi: 10.1097/MPH.0000000000000755.
Secondary hemophagocytic lymphohistiocytosis (sHLH) is a rare hyperinflammatory disorder caused by an aberrant immune response to a number of infectious or inflammatory conditions. Successful treatment of this potentially fatal condition requires early recognition and prompt therapy directed at the underlying trigger. In this report, we describe the clinical presentation, diagnostic findings, management, and outcome of a child with Lemierre's syndrome-associated sHLH. This is the first reported association of these 2 rare conditions and expands the number of known triggers for sHLH.
继发性噬血细胞性淋巴组织细胞增生症（sHLH）是一种罕见的高炎症性疾病，由对多种感染或炎症状态的异常免疫反应引起。成功治疗这种潜在致命疾病需要早期识别并针对潜在触发因素进行及时治疗。在本报告中，我们描述了一名患有勒米尔综合征相关sHLH的儿童的临床表现、诊断结果、治疗及预后。这是这两种罕见疾病首次报道的关联，扩大了已知的sHLH触发因素的范围。
