Sung L, Weitzman S S, Petric M, King S M
Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada.
Clin Infect Dis. 2001 Nov 15;33(10):1644-8. doi: 10.1086/323675. Epub 2001 Oct 5.
There is a paucity of literature addressing infection-related morbidity and mortality in children with primary hemophagocytic lymphohistiocytosis (HLH), a rare condition characterized by abnormal proliferation of macrophages, hypercytokinemia, and T cell immunosuppression. Therefore, a retrospective chart review was done of patients diagnosed with primary HLH over a 15-year period. Significant infections present at diagnosis, during the course of illness, and just prior to death or at autopsy were noted. Of the 18 children identified with primary HLH, an infectious agent was documented at the initial presentation of HLH in 5. Significant infections occurred during therapy in 10 (56%) of 18. Of the 12 fatal cases, invasive infection was the cause of death in 8 children, and 6 of these deaths were directly attributable to invasive fungal infection. Significant infections were common during therapy in children with primary HLH, and fungal infections were an important cause of mortality in this group.
关于原发性噬血细胞性淋巴组织细胞增生症（HLH）患儿感染相关的发病率和死亡率的文献较少，HLH是一种罕见疾病，其特征为巨噬细胞异常增殖、高细胞因子血症和T细胞免疫抑制。因此，对15年间诊断为原发性HLH的患者进行了回顾性病历审查。记录了诊断时、病程中、死亡前或尸检时出现的严重感染。在确诊为原发性HLH的18名儿童中，5名在HLH初次就诊时有感染病原体记录。18名患儿中有10名（56%）在治疗期间发生严重感染。在12例死亡病例中，侵袭性感染是8名儿童的死亡原因，其中6例死亡直接归因于侵袭性真菌感染。原发性HLH患儿在治疗期间严重感染很常见，真菌感染是该组患儿死亡的重要原因。
