Palazzi Debra L, McClain Kenneth L, Kaplan Sheldon L
Infectious Diseases Section, Department of Pediatrics, Baylor College of Medicine and Texas Children's Hospital, Houston, TX 77030, USA.
Clin Infect Dis. 2003 Feb 1;36(3):306-12. doi: 10.1086/345903. Epub 2003 Jan 14.
To study the evolution of hemophagocytic syndrome (HPS) in children, we performed a retrospective review of 19 patients (median age, 17.4 months) in whom an infectious diseases consultation was requested at Texas Children's Hospital during the period of September 1991 through September 2001. Clinical findings consistent with HPS most frequently presented during days 6-14 of illness, concomitant with laboratory abnormalities. Fever was present for a median of 19 days before the diagnosis of HPS. Elevated serum lactate dehydrogenase and ferritin levels were noted in all patients. An infectious agent was identified in 42% of patients; 16% were found to have immunologic or vasculitic disease. HPS is a rare but often fatal disease that can initially present as fever of unknown origin with varying clinical findings, and it can be recognized by physicians who are familiar with the evolution of HPS. It is likely that many of these cases remain undiagnosed because of the HPS's rapidly fatal course.
为研究儿童噬血细胞综合征（HPS）的演变，我们对1991年9月至2001年9月期间在德克萨斯儿童医院因感染性疾病会诊的19例患者（中位年龄17.4个月）进行了回顾性研究。与HPS一致的临床发现最常出现在病程的第6至14天，并伴有实验室异常。在诊断HPS之前，发热的中位时间为19天。所有患者均发现血清乳酸脱氢酶和铁蛋白水平升高。42%的患者鉴定出感染因子；16%的患者被发现患有免疫性或血管炎性疾病。HPS是一种罕见但通常致命的疾病，最初可能表现为不明原因发热及各种临床症状，熟悉HPS演变过程的医生能够识别该病。由于HPS病程进展迅速且致命，很可能许多此类病例仍未得到诊断。
