Clinical Diagnosis And Treatment Of Children With Hemophagocytic Lymphohistiocytosis

Objectives: To explore the clinical characteristics, etiology and clinical treatment of hemophagocytic lymphohistiocytosis(HLH), in order to improve the clinical diagnosis and treatment of the disease, by retrospectively analyzing diagnosed HLH in department of pediatrics, people’s hospital of Xinjiang Uygur autonomous region for the past 10 years. Methods: Retrieval the 48 cases of children with HLH in department of pediatrics, people’s hospital of Xinjiang Uygur autonomous region from January 2006 to January 2016, and summarize the clinical features, laboratory indexes, therapeutic method and prognosis, then, analyze the data. Results:(1) In 48 HLH, the male and female children are both 24, and the peak age is 3 months to 2 years old, the main reason is autumn and winter. All the patients had fever, splenomegaly and three series reduction, 60.4% of the patients with respiratory symptoms. Liver damage is mainly increased ALT(93.6%), AST(97.9%) and LDH(97.9%). The proportion of PT(97.7%) and APTT prolongation(79.1%), ferritin(93.8%), serum Na(85.4%) and TG increased(66.8%), ALB(90.9%) and Fib decreased(81.3%) is also high, while that of CREA(4.9%) and BUN increased(7.3%) is low.(2) In 48 HLH, 36 of them are secondary HLH, 22 were leishmaniasis associated HLH, accounting for 45.2%(22/48). The main treatment of leishmaniasis associated HLH were sodium stibogluconate and dexamethasone, 16 were cured, 6 discharged voluntarily or died. Brucellosis, tuberculosis and enterococcus associated with HLH was 1 case respectively, which were cured by treatment of primary disease. While 1 Staphylococcus aureus associated HLH and 1 Staphylococcus epidermidis infection associated HLH died, although they were treated by anti-infection and other methods. 8 cases with unknown pathogens were treated with anti-inflammatory and anti-infection, of them, 4 were cured, 4 died or discharged voluntarily. 1 leukemia associated HLH got better with chemotherapy. In 12 HLH with unknown etiology, 2 died,10 discharged voluntarily.(3) 48 HLH were divided into two groups,including leishmaniasis associated HLH and non leishmaniasis associated HLH. Compared two groups with the epidemiological data, laboratory indexes and prognostic features. The age, sex, race, Hb, WBC, ALB, TBIL, TG, PT, APTT, Fib and serum sodium of the two groups had no statistical difference(P>0.05);regional, seasonal, Plt, AST, ALT, LDH, prognosis of the two groups had statistical difference(P<0.05). Conclusions: The etiology and clinical manifestations of children HLH are diversity, most of them are infants and young children. All patients have fever, splenomegaly and pancytopenia, most of the patients have liver damage and respiratory system involvement. In our study, most of the HLH were secondary HLH, and most of the secondary HLH were leishmaniasis associated HLH. In autumn and winter, the HLH children who come from the south of Xinjiang should be given more attention if they are leishmaniasis associated HLH. The liver damage of leishmaniasis associated HLH is more serious than that of non leishmaniasis associated HLH. If the secondary HLH is diagnosed timely, effect of therapy and prognosis will be better. The hospitals should establish a method to test the primary HLH, in order to aid clinical diagnosis in Xinjiang.