A Analysis Of Clinical Characteristics Of 26 Cases With Hemophagocytic Syndrome

Objective:To analyze the clinical characteristics and laboratory characteristics of the HPS.Methods:A retrospective study was conducted with HPS in preliminary diagnosis of our center between September 2010 and September 2015.Results:The clinical data of 26 newly diagnosed patients with HPS(since September 2010 to September 2015) was enrolled by a retrospective study from The first hospital of Lanzhou university. Among the 26 newly diagnosed HPS patients, the ratio of male to female was 1.17:1(14 male and 12 female). There are 5 primary and 21 secondary HPS in the 26 cases. The median age was 16.5 years old(range 0.48-6 years). The median age of primary HPS patients is 2 years old(4.74±4.835y)compared with the 31 years old(32.87±25.555y)of the secondary HPS patients. The HPS secondary to infection account for 76.19%(16/21), including 6 cases of Epstein-barr virus infection(37.5%), 3 cases of fungal infections(18.75%), 3 cases of amastigote parasite infection(18.75%). There 19.05% secondary to the lymphoma(4 /21). The common symptoms of the 26 cases are unremitting fever(96.15%), splenomegaly(65.38%), hepatomegaly(38.46%), bleeding(19.23%), lymph node enlargement(19.23%)and skin rash(15.38%). Laboratory tests reveal the most common feature are decreased peripheral blood cells(100%) and elevated liver transaminases(92.31%), the elevated ferritine(90.91%), decreased albumin(69.23%), decreased fibrinogen(53.85%), elevated lactate dehydrogenase(46.15%), elevated total bilirubin(42.31%),elevated triglyceride(30.77%) followly. Hemophagocytosis in bone marrow takes up 69.57%. The median of blood cell is as follows: WBC 1.37(0.41~11.25×109), HB 85(46~147)g/L, PLT 37(1~250×109). The fever and splenomegaly are in the highest flight of positive rate of diagnostic indices(respectively 96.15%, 65.38%). There are 16 death and 10 survival in the 26 cases(death vs survival=1.6:1). 4 cases died in the primary HPS(fatality rate: 80%). 12 cases died in the secondary HPS(fatality rate: 57.14%). The overall fatality rate is 61.54 %. There was no statistically significant difference in peripheral blood leucocytes, transaminase, triglyceride, fibrinogen, bilirubin, ferritin and lactic dehydrogenase(P>0.05).Conclusion:1. The HPS has the feature of acute onset, speedy development and high death rate. The HPS should be considered if patients mainly conduct unremitting fever, splenomegaly and lymph node enlargement, meanwhile the laboratory tests reveal the decreased peripheral blood cells, elevated liver transaminases, decreased fibrinogen, elevated triglyceride. You can test the bone marrow, the lactate dehydrogenase, the sCD25, the ferritine and the activity of NK cells.2. The levels of peripheral blood leucocytes, transaminase, triglyceride, fibrinogen, bilirubin, ferritin and lactic dehydrogenase of patients have not relationship with the prognosis.