Characteristics Of Fifty Chinese Cases With Hemophagocytic Lymphohistiocytosis

Objective To identify clinical and/or laboratory features of hemophagocytic lymphohistiocytosis (HLH) in a Chinese population, to improve early diagnosis and treatment intervention of HLH, and to reduce mortality of HLH.Methods A retrospective analysis of50diagnosed HLH cases who were hospitalized from year2008to2013in the Second Hospital of Shandong University and Linyi People’s Hospital was carried out. All their medical records were reviewed and analyzed. For each patient, demographic, clinical, laboratory, imaging data and the response to different treatments were collected. According to HLH-2004diagnostic criteria the50diagnosed patients were divided into two groups: adults’ group and pediatric group.Results(1) Etiology: Adult non-viral associated HLH proportion was higher, but children had high proportion of EB virus associated HLH.(2) The diagnosed patients were clinically characterized by continuous or intermittent fever, splenomegaly, hepatomegaly, respiratory system involvement, jaundice, bleeding and hy drops of serous cavity.(3) Laboratory tests indicated that the patients had elevated serum ferritin, impaired liver function, peripheral blood cytopenias, hemophagocytic phenomenon in bone marrow, as well as elevated C-reactive protein, and increase lactate dehydrogenase, followed by hyponatremia, hypocalcemia and hypertriglyceridemia and so on.(4)Comparison of adults and pediatric group:pediatric group in the splenomegaly(P=0.002), hepatomegaly(P=0.011) and jaundice(P=0.002) was more obvious than in adults; Laboratory data in children showed more severe reduction in hemoglobin(P=0.002) and increased serum total bilirubin(P=0.001), alanine aminotransferase (P=0.023), lacate dehydrogenase (P=0.018)etc. than in adults.(5) Response to different treatments:the serum ferritin levels and lactate dehydrogenase levels decreased significantly than before treatment of the two groups(chemotherapy group and the non-chemotherapy group) after two weeks’treatment(P≤0.01). Chemotherapy group also showed significantly higher platelet count after2～3weeks’ therapy (P=0.032), but the non-chemotherapy group was not.Conclusions(1) There were various and multiplex clinical manifestations and etiologies. When patients presented with long-term fever, hepatosplenomegaly, cytopenia, liver dysfunction, significantly high lacate dehydrogenase and ferritin, they should be highly suspected with HLH.(2) Adult and children had their own characteristics of clinical presentation and laboratory data, we should seek early diagnosis, early treatment, reduce misdiagnosis and mortality.