CAJ | 학술논문

目的分析嗜血细胞综合征(HPS)的临床特点,探讨发病原因、诊断及治疗,提高临床医疗水平.方法对江苏省人民医院2000年1月至2007年7月收治的12例HPS患者进行回顾性分析,并复习相关文献.结果 12例患者均为继发性HPS,多继发于病毒和细菌感染,亦有系统性红斑狼疮和坏死性淋巴结炎.均出现高热,在短时间内三系细胞减少,肝功能异常,经抗病毒、抗感染等原发病的积极治疗和对症支持治疗后,11例患者好转出院,1例死亡.结论 HPS发病率低,预后较差.故对于不明原因的发热、三系细胞减少患者需多次多部位进行骨髓检查,以求尽早诊断,合理有效治疗,改善预后.
목적 분석기혈세포종합정(HPS)적림상특점,탐토발병원인、진단급치료,제고림상의료수평.방법 대강소성인민의원2000년1월지2007년7월수치적12례HPS환자진행회고성분석,병복습상관문헌.결과 12례환자균위계발성HPS,다계발우병독화세균감염,역유계통성홍반랑창화배사성림파결염.균출현고열,재단시간내삼계세포감소,간공능이상,경항병독、항감염등원발병적적겁치료화대증지지치료후,11례환자호전출원,1례사망.결론 HPS발병솔저,예후교차.고대우불명원인적발열、삼계세포감소환자수다차다부위진행골수검사,이구진조진단,합리유효치료,개선예후.
Objective To enhance the understanding of hemophagocytic syndrome(HPS)by analyzing the clinical manifestations, diagnosis and therapy. Methods The clinical data of 12 patients with HPS were retrospectively collected in the People's Hospital of Jiangsu Province from 2000 to 2007. The relevant literature were reviewed. Results Twelve patients were diagnosed as secondary hemophagocytic syndrome most secondary to virus and bacteria infection. Some patients condition was associated with systemic lupus erythematosus or histiocytic necrotizing lympheadenitis. All of the 12 patients had high fever, abnormal liver function and showed a decrease in the number of blood cells in a short time. After antivirus and antibiotic treatment, 11 patients'condition were improved and 1 patient died. Conclusion Hemophagocytic syndrome is not a common clinical condition but with poor prognosis. When patient presents with fever without apparent reasons and pancytopenia, bone marrow examination should be done and sometimes repeated bone marrow examinations are needed. The diagnosis of secondary haemophagocytic syndrome needs multidisciplineary cooperation. Aggressive diagnostic procedures are needed to clarify the diagnosis and prompt treatments are warranted to improve prognosis.