Abstract：OBJECTIVE: To study the main factors influencing prognosis of hemophagocytic lymphohistiocytosis (HLH) in children by summarizing the clinical features of HLH and investigating the relationship between relevant factors and prognosis. METHODS: The medical data of 63 children with HLH were retrospectively reviewed. Kaplan-Meier method was employed to draw survival curves. Factors influencing prognosis were assessed with Cox univariate analysis, and Cox multivariate analysis was done on statistically significant factors. RESULTS: The 3-year and 5-year survival rates were both 62.9%. The survival rate decreased from 98.4% at 1 day after definite diagnosis to 73.2% at 4 months. Univariate analysis demonstrated only one factor, which was that the condition of platelet recovery after treatment of 2 to 3 weeks was significantly related to prognosis (P=0.002). In children receiving etoposide therapy, temperature recovery after one day of treatment was significantly related to prognosis (P=0.016). CONCLUSIONS: Children with HLH have a satisfactory prognosis, but the survival rate reduces rapidly in the first 4 months after definite diagnosis. Platelet recovery after treatment of 2 to 3 weeks and temperature recovery after one day of treatment are factors influencing prognosis of HLH in children.
［1］Gholam C, Grigoriadou S, Gilmour KC, Gaspar HB. Familial haemophagocytic lymphohistiocytosis: advances in the genetic basis, diagnosis and management［J］. Clin Exp Immunol, 2011, 163(3): 271-283.
［2］Henter JL, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis［J］. Pediatr Blood Cancer, 2007, 48(2): 124-131.
［3］Henter JI, Elinder G, Soder O, Ost A. Incidence in Sweden and clinical features of familial hemophagocytic lymphohistiocytosis［J］. Acta Paediatr Scand, 1991, 80（4）: 428-435.
［7］Trottestam H, Horne A, Aricò M, Egeler RM, Filipovich AH, Gadner H, et al. Chemoimmunotherapy for hemophagocytic lymphohistiocytosis: long-term results of the HLH-94 treatment protocol［J］. Blood, 2011, 118(17): 4577-4584.
［9］Ramachandran B, Balasubramanian S, Abhishek N, Ravikumar KG, Ramanan AV. Profile of hemophagocytic lymphohistiocytosis in children in a tertiary care hospital in India［J］. Indian Pediatr, 2011, 48(1): 31-35.
［10］Kim HK, Kim HG, Cho SJ, Hong YM, Sohn S, Yoo ES, et al. Clinical characteristics of hemophagocytic lymphohistiocytosis related to Kawasaki disease［J］. Pediatr Hematol Oncol, 2011, 28(3): 230-236.
［12］Janka GE, Schneider EM. Modern management of children with haemophagocytic lymphohistiocytosis［J］. Br J Haematol, 2004, 124(1): 4-14.
［13］Maakaroun NR, Moanna A, Jacob JT, Albrecht H. Viral infections associated with haemophagocytic syndrome［J］. Rev Med Virol, 2010, 20(2): 93-105.
［14］Tang YM, Xu XJ. Advances in hemophagocytic lymphohistiocytosis: pathogenesis, early diagnosis/differential diagnosis, and treatment［J］. Sci World J, 2011, 11(3): 697708.
［15］Imashuku S, Hyakuna N, Funabiki T, Ikuta K, Sako M, Iwai A, et al. Low natural killer activity and central nervous system disease as a high-risk prognostic indicator in young patients with hemophagocytic lymphohistiocytosis［J］. Cancer, 2002, 94(11): 3023-3031.
［17］Horne A, Trottestam H, Aricò M, Egeler RM, Filipovich AH, Gadner H, et al. Frequency and spectrum of central nervous system involvement in 193 children with haemophagocytic lymphohistiocytosis ［J］. Br J Haematol, 2008, 140(3): 327-335.
［18］Ishii E, Ohga S, Imashuku S, Yasukawa M, Tsuda H, Miura I, et al. Nationwide survey of hemophagocytic lymphohistiocytosis in Japan［J］. Int J Hematol, 2007, 86(1): 58-65.
［20］Imashuku S, Teramura T, Tauchi H, Ishida Y, Otoh Y, Sawada M, et al. Longitudinal follow-up of patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis［J］. Haematologica, 2004, 89(2): 183-188.
［21］Imashuku S. Clinical features and treatment strategies of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis［J］. Crit Rev Oncol Hematol, 2002, 44(3): 259-272.
