The role of infections in primary hemophagocytic lymphohistiocytosis: a case series and review of the literature.
The role of infections in primary hemophagocytic lymphohistiocytosis: a case series and review of the literature.
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DOI：
10.1086/323675
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发表时间：
2001-11
期刊：
影响因子：
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通讯作者：
L. Sung;S. Weitzman;M. Petrič;S. King;L. Sung
中科院分区：
文献类型：
--
作者：
L. Sung;S. Weitzman;M. Petrič;S. King;L. Sung
关于原发性噬血细胞性淋巴组织细胞增生症（HLH）儿童感染相关发病率和死亡率的文献很少，HLH是一种罕见的疾病，其特征是巨噬细胞异常增殖、高细胞因子血症和T细胞免疫抑制。因此，对15年内诊断为原发性HLH的患者进行了回顾性病历审查。在诊断时、病程中、死亡前或尸检时观察到严重感染。在18例原发性HLH患儿中，有5例在HLH最初表现时记录了感染原。18例患者中有10例（56%）在治疗期间发生了严重感染。在12例死亡病例中，侵袭性感染是8例儿童死亡的原因，其中6例死亡直接归因于侵袭性真菌感染。治疗期间，原发性HLH患儿常见严重感染，真菌感染是该组死亡的重要原因。
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.