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