Prognostic factors for hemophagocytic lymphohistiocytosis in children

LU Wen-Xian, LUO Jian-Ming

Chinese Journal of Contemporary Pediatrics ›› 2012, Vol. 14 ›› Issue (08) : 593-597.

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Chinese Journal of Contemporary Pediatrics ›› 2012, Vol. 14 ›› Issue (08) : 593-597.
CLINICAL RESEARCH

Prognostic factors for hemophagocytic lymphohistiocytosis in children

  • LU Wen-Xian, LUO Jian-Ming
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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.

Key words

Hemophagocytic lymphohistiocytosis / Prognosis / Child

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LU Wen-Xian, LUO Jian-Ming. Prognostic factors for hemophagocytic lymphohistiocytosis in children[J]. Chinese Journal of Contemporary Pediatrics. 2012, 14(08): 593-597

References

[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.

[4]Janka GE. Familial hemophagocytic lymphohistiocytosis[J]. Eur J Pediatr, 1983, 140(3): 221-230.

[5]Janka G, Imashuku S, Elinder G, Schneider M, Henter JI. Infection-and malignancy-associated hemophagocytic syndromes. Secondary hemophagocytic lymphohistiocytosis[J]. Hematol Oncol Clin North Am, 1998, 12(2): 435-444.

[6]广东省小儿噬血细胞综合征协作组.儿童噬血细胞综合征68例临床研究[J]. 中国小儿血液与肿瘤杂志, 2009, 14(1): 20-22.

[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.

[8]Janka GE. Familial and acquired hemophagocytic lymphohistiocytosis[J]. Eur J Pediatr, 2007, 166(2): 95-109.

[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.

[11]石杨,王丽杰.小儿噬血细胞综合征临床特点及预后危险因素分析[J]. 中国现代医学杂志, 2011, 21(5):645-651.

[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): 697708.

[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.

[16]王冠玲,胡群,张柳清,刘爱国,刘双又,张耀东,等.儿童噬血细胞综合征死亡相关因素分析[J].临床儿科杂志,2010,28(5):438-441.

[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.

[19]金颖康,谢正德,杜忠东,杨双,申昆玲.儿童Epstein-Bar病毒相关性噬血细胞性淋巴组织细胞增生症的回顾性分析[J].首都医科大学学报, 2010, 31(2):192-196.

[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.
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