吉林大学学报（医学版）2016，Vol.42Issue(6)：1197-1202,6.DOI:10.13481/j.1671-587x.20160629
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
Analysis of clinical characteristics of patients with adult-onset Still’s disease complicated with hemophagocytic syndrome:A report of 3 cases and literature review
摘要
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.关键词
成人斯蒂尔病/噬血细胞综合征/血小板/丙氨酸氨基转移酶Key words
adult onset Still’s disease/hemophagocytic syndrome/platelet/alanine aminotransferase分类
医药卫生引用本文复制引用
高旗,袁艺,马宁,姜振宇..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[J].吉林大学学报（医学版）,2016,42(6):1197-1202,6.基金项目
吉林省科技厅科技发展计划项目资助课题 （）
