REACTIVE HEMOPHAGOCYTIC SYNDROME - A CLINICOPATHOLOGICAL STUDY OF 40 PATIENTS IN AN ORIENTAL POPULATION
REACTIVE HEMOPHAGOCYTIC SYNDROME - A CLINICOPATHOLOGICAL STUDY OF 40 PATIENTS IN AN ORIENTAL POPULATION
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DOI：
10.1016/0002-9343(92)90048-g
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发表时间：
1992-08-01
影响因子：
5.9
通讯作者：
CHAN, JKC
中科院分区：
文献类型：
--
作者：
WONG, KF;CHAN, JKC
目的：本次调查的目的是评估东方人群中反应性噬血细胞综合征 (RHS) 的临床谱和结果。 患者和方法： 这项调查是对香港社区医院伊丽莎白医院 40 名连续 40 名在 4 年内诊断为 RHS 的患者进行的回顾性研究。 结果：香港 RHS 的发病率似乎与西方国家相当。然而，与 RHS 相关的疾病范围明显不同。百分之四十的病例与恶性淋巴瘤有关。另外 40% 的病例与感染有关，通常是由细菌引起的，而由疱疹病毒引起的感染并不常见，这可能反映了与白人的种族和地理差异。两名患者患有急性狼疮噬血细胞综合征，这是一种独特的 RHS 形式，发生在系统性红斑狼疮患者中。治疗基本上是支持性的，如果存在基础疾病，也会给予特定的治疗。 18 名患者（45%）死于急性疾病，大部分是由于 RHS 并发症或基础疾病所致。 发生播散性血管内凝血病是一个不良预后因素。结论：RHS 在香港是一种罕见疾病，通常与恶性淋巴瘤或细菌感染有关。因此，一旦做出诊断，就需要严格寻找潜在的恶性肿瘤或感染。
PURPOSE: The purpose of this investigation was to assess the clinical spectrum and outcome of reactive hemophagocytic syndrome (RHS) in an Oriental population.PATIENTS AND METHODS: The investigation was a retrospective study of 40 consecutive patients with RHS diagnosed over a 4-year period in Queen Elizabeth Hospital, a community based hospital in Hong Kong.RESULTS: The incidence of RHS m Hong Kong appeared to be comparable with that of Western countries. However, the spectrum of diseases associated with RHS was distinctly different. Forty percent of the cases were associated with malignant lymphoma. Another 40% of the cases were associated with infection, often due to bacteria, and infection due to the herpes group of viruses was uncommon, probably reflecting ethnic and geographic differences from the white population. Two patients had the acute lupus hemophagocytic syndrome, a distinctive form of RHS that occurs in patients with systemic lupus erythematosus. Treatment was basically supportive, with specific therapy also given for the underlying disease if present. Eighteen patients (45%) died of the acute disease, mostly as a result of complications of RHS or the underlying diseases. The development of disseminated intravascular coagulopathy was a poor prognostic factor.CONCLUSIONS: RHS is an uncommon disorder in Hong Kong and is often associated with malignant lymphoma or bacterial infection. A rigorous search for an underlying malignancy or infection is thus warranted once this diagnosis is made.