吉林大学学报(医学版) ›› 2016, Vol. 42 ›› Issue (06): 1197-1202.doi: 10.13481/j.1671-587x.20160629

• 临床医学 • 上一篇    下一篇

3例成人斯蒂尔病并发噬血细胞综合征患者的临床特点分析并文献复习

高旗, 袁艺, 马宁, 姜振宇   

  1. 吉林大学第一医院风湿免疫科, 吉林 长春 130021
  • 收稿日期:2016-04-19 出版日期:2016-11-28 发布日期:2016-12-02
  • 通讯作者: 姜振宇,教授,主任医师,博士研究生导师(Tel:0431-88782060,E-mail:jlujzy@aliyun.com) E-mail:jlujzy@aliyun.com
  • 作者简介:高旗(1990-),女,河北省唐山市人,医师,在读医学硕士,主要从事风湿免疫性疾病的基础和临床方面的研究。
  • 基金资助:

    吉林省科技厅科技发展计划项目资助课题(20150101152JC)

Analysis of clinical characteristics of patients with adult-onset Still's disease complicated with hemophagocytic syndrome: A report of 3 cases and literature review

GAO Qi, YUAN Yi, MA Ning, JIANG Zhenyu   

  1. Department of Rheumatology, First Hospital, Jilin University, Changchun 130021, China
  • Received:2016-04-19 Online:2016-11-28 Published:2016-12-02

摘要:

目的:探讨成人斯蒂尔病(AOSD)并发噬血细胞综合征(HPS)患者的临床表现、实验室指标特点和治疗预后情况,为其临床诊治提供依据。方法:收集3例AOSD并发HPS患者的性别、年龄、发热峰值温度、皮疹、关节肿痛、肝脾淋巴结肿大及治疗和预后情况等临床数据以及外周血白细胞(WBC)数量、中性粒细胞绝对值(NE#)、淋巴细胞绝对值(LY#)、单核细胞绝对值(MO#)、红细胞(RBC)、血红蛋白(HGB)、血小板(PLT)、天门冬氨酸氨基转移酶(AST)、谷丙氨酸氨基转移酶(ALT)、谷氨酰转肽酶(GGT)、碱性磷酸酶(ALP)、胆碱酯酶(CHE)、血清铁蛋白(SF)、纤维蛋白原(FBG)及骨髓象等实验室数据,并对其进行回顾性分析。结果:一般情况,3例患者均为50岁以上女性,出现HPS的时间分别为AOSD发病后的1个月、1个月和5个月。临床表现,3例AOSD患者均以发热、皮疹和关节疼痛为首发症状,均无肝脾肿大,仅患者3在AOSD初期出现淋巴结肿大,经糖皮质激素治疗后发热、皮疹、关节肿痛及淋巴结肿大症状均好转。并发HPS后3例患者均再次首先出现发热症状,体温均在39℃以上,其中2例患者出现黄疸,出现HPS后均未发现肝脾及淋巴结肿大。实验室指标特点,血细胞减少和持续升高的SF为AOSD并发HPS的早期敏感指标,肝功能酶学及FBG水平的变化均滞后于血细胞和SF变化。AOSD并发HPS患者肝功能异常以ALT水平升高最为明显。血细胞减少早期行骨穿活检即可发现吞噬现象。结论:AOSD并发HPS患者的临床特点为不明原因的再发持续高热,实验室指标的特点为血细胞减少(尤其是血小板减少)、SF持续升高、重度肝损伤(以ALT水平升高为主)、纤维蛋白原减少和骨穿活检出现吞噬现象;AOSD并发HPS患者应早发现、早治疗,以降低其复发率及死亡率。

关键词: 成人斯蒂尔病, 噬血细胞综合征, 丙氨酸氨基转移酶, 血小板

Abstract:

Objective: To explore the clinical characteristics,laboratory indicator features,treatment,and prognosis of the patients with adult-onset Still's disease(AOSD) complicated with hemophagocytic syndrome(HPS),and to provide basis for its diagnosis and treatment.Methods: The clinical data including age,gender,peak of temperature,rash,joint swelling,polyarthralgia,lymphadenopathy,hepatosplenomegaly,and the treatment and prognosis,and the laboratory data including the absolute value of peripheral white blood cells (WBC),neutrophills (NE#),lymphocytes (LY#),monocytes (MO#) and red blood cells (RBC),hemoglobin (HGB),platelet (PLT),aspartate aminotransferase (AST),alanine aminotransferase (ALT), glutamyl-transpeptidase (GGT),alkaline phosphatase (ALP),cholinesterase (CHE),serum ferritin (SF),fibrinogen (FBG),myelogram of three patients with AOSD complicated with HPS were collected and retrospectively analyzed.Results: The general situation showed that all of three patients were female aged over 50 years old. The onset time of HPS was 1 month,1 month and 5 months after AOSD,respectively. All the patients presented fever,rash,and arthritis as the initial symptoms without hepatomegaly or splenomegaly.Only No.3 patient presented lymphadenophy at the beginning of AOSD.After glucocorticoid treatment,the symptoms such as fever,rash,arthritis,and lymphadenophy were improved.The initial symptom of AOSD patient after complicated with HPS was fever,and the temperature was over 39℃.Two patients presented jaundice,no one presented hepatomegaly,splenomegaly or lymphadenophy.The laboratory test results showed that the cytopenia and presistently increasing SF were the early sensitive indicators of AOSD complicated with HPS.The changes of liver enzymes and FBG level followed behind the changes of blood cells and SF level. The phagocytosis was found when bone marrow puncture was performed at the early stage of cytopenia.Conclusion: The clinical characteristic of AOSD patients complicated with HPS is an unexplainedly,recurrently, and continuously high fever,and the characteristics of laboratory test are cytopenia(especially thrombocyopenia),the persistently increasing SF,severe liver injury(especially the increased ALT level),hypofibrinogenemia, and phagocytosis found in bone marrow puncture. The AOSD patients should be early diagnosed and treated in order to reduce the relapse rate and mortality rate.

Key words: hemophagocytic syndrome, platelet, adult onset Still's disease, alanine aminotransferase

中图分类号: 

  • R58