The Clinical Features Of Hemophagocytic Lymphohistiocytosis

Objective: To Investigate the clinical features,risk factors and rational administrations of hemophagocytic lymphohistiocytosis(HLH).Methods: The medical records of 23 HLH cases hospitalized in Chilren,s Hospital affiliated to Chongqing University of Medical Science from November 2004 to January 2007,were reviewed and the clinical features were analyzed.Results: (1)The ratio of boys versus girls was 1.875:1, 65.2% of the patients were aged under 5 years old.(2)The main features of HLH were fever,splenohepatomegalia,lymphadenectasis and jaundice er al. The central nervous system(CNS) symptoms were unusual. The hemorrhagic tendency are common in the course, but they were rare at the beginning of the disease.(3)Besides the typical features such as pancytopenia, hypertriglyceridemia and/or hypofibrinogenemia, Hyperferritinemia, decreased NK-cell activity and hemophagocytosis in bone marrow, the abnormallities of hepatic function and immune system are striking( elevated transaminases, bilirubin and LDH ;degraded IgA, IgM in the serum e.g.)(4).Dividing the cases into improved group and depraved group,we found that the difference of CD19,PT,Fib,C4 and VP-16 between the two groups need further investigation (0.05