Hemophagocytic Lymphohistiocytosis with Erythra as an Outstanding Manifest: A Case Report and the Literature Review_West China Medical Journal

Authors：

LI Hui , XU Ming , ZHOU Min , LI Dan , SHI Hong

Department of Hematology and Oncology, Chengdu Women and Children Central Hospital, Chengdu, Sichuan 610019, P. R. China;

Corresponding author：

Keywords：

噬血细胞综合征; 皮疹

DOI：

CNKI: 51-1356/R.20120214.1338.008

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目的　总结1例噬血细胞综合征的临床表现及治疗方案。方法　 2009年12月收治1例以皮疹为突出表现的噬血细胞综合征患儿的临床表现及治疗效果。结果　患儿的皮疹表现突出，临床较少见；采用HLH-2004方案治疗后临床症状、体征及实验室指标短时间内恢复正常。近期随访，一般情况良好，血液学指标基本正常。结论　患儿采用HLH-2004方案治疗后预后良好。

Citation： LI Hui,XU Ming,ZHOU Min,LI Dan,SHI Hong. Hemophagocytic Lymphohistiocytosis with Erythra as an Outstanding Manifest: A Case Report and the Literature Review. West China Medical Journal, 2012, 27(2): 182-184. doi: CNKI: 51-1356/R.20120214.1338.008 Copy

1.	Farquhar JW, Claireaux AE. Familial haemophagocytic reticulosis[J]. Arch Dis Child, 1952, 27(136): 519-525.
2.	Horne A, Ramme KG, Rudd E, et al. Characterization of PRF1, STX11 and UNC13D genotype -phenotype correlations in familial hemophagocytic lymphohistiocy tosis[J]. Br J Haematol, 2008, 143(1): 75-83.
3.	Ishii E, Ohga S, Imashuku S, Imashuku S, et al. Nationwide survey of hemophagocytic lymphohistiocytosis in Japan[J]. Int J Hematol, 2007, 86(1): 58-65.
4.	Henter JI, Horne A, Arico M, et al. HLH-2004: diagnos tic and therapeutic guidelines for hemophagocytic lymphohistiocytosis[J]. Pediatr Blood Cancer, 2007, 48(2): 124-131.
5.	广东省小儿噬血细胞综合症协作组. 儿童噬血细胞综合症68例研究[J]. 中国小儿血液与肿瘤杂志, 2009, 14(1): 20-22.
6.	刘苗，徐佳伟，王艳荣, 等.儿童噬血细胞综合症28例临床特点与预后分析[J]. 临床儿科杂志, 2009, 27(8): 727-730.
7.	Janka GE. Familial hemophagocytic lymphohistiocytosis[J]. Eur J Pediatr, 1983, 140(3): 221-230.
8.	Zhou Y, Gao YJ, Zhu XH, et al. Diagnosis and treatment of hemophagocytic lymphohistiocytosis in children with HLH-2004 protocol[J]. J Clin Pediatr, 2009, 21(8): 221-224.
9.	Janka GE, Schneider EM. Modern management of children with haemophagocytic lymphohistiocytosis[J]. Br J Haematol, 2004, 124(1): 4-14.
10.	Janka GE. Hemophagocytic syndromes[J]. Blood Rev, 2007, 21(5): 245-253.
11.	薛红漫, 岑丹阳, 夏焱, 等. 噬血细胞综合征-04方案治疗小儿EB病毒相关性噬血细胞综合征[J]. 中华妇幼临床医学杂志, 2009, 5(3): 56-59.
12.	Imashulcu S, Kuriyama K, Teramura T, et al. Requirement for Etoposide in the treatment of Epstem-Barr virus associated hemophagocytic lymphohistioeytosis[J]. J clm Onool, 2001, 19(10): 2665-2673.
13.	李欣, 徐酉华. 18例EB病毒相关性噬血淋巴组织细胞增生症的临床分析[J]. 中国小儿血液与肿瘤杂志, 2009, 14(3): 131-133.
14.	朱音, 高怡瑾, 朱晓华, 等. HLH-2004方案诊断治疗小儿噬血细胞综合征结果分析[J]. 临床儿科杂志, 2009, 27(8): 718-722.

1. Farquhar JW, Claireaux AE. Familial haemophagocytic reticulosis[J]. Arch Dis Child, 1952, 27(136): 519-525.
2. Horne A, Ramme KG, Rudd E, et al. Characterization of PRF1, STX11 and UNC13D genotype -phenotype correlations in familial hemophagocytic lymphohistiocy tosis[J]. Br J Haematol, 2008, 143(1): 75-83.
3. Ishii E, Ohga S, Imashuku S, Imashuku S, et al. Nationwide survey of hemophagocytic lymphohistiocytosis in Japan[J]. Int J Hematol, 2007, 86(1): 58-65.
4. Henter JI, Horne A, Arico M, et al. HLH-2004: diagnos tic and therapeutic guidelines for hemophagocytic lymphohistiocytosis[J]. Pediatr Blood Cancer, 2007, 48(2): 124-131.
5. 广东省小儿噬血细胞综合症协作组. 儿童噬血细胞综合症68例研究[J]. 中国小儿血液与肿瘤杂志, 2009, 14(1): 20-22.
6. 刘苗，徐佳伟，王艳荣, 等.儿童噬血细胞综合症28例临床特点与预后分析[J]. 临床儿科杂志, 2009, 27(8): 727-730.
7. Janka GE. Familial hemophagocytic lymphohistiocytosis[J]. Eur J Pediatr, 1983, 140(3): 221-230.
8. Zhou Y, Gao YJ, Zhu XH, et al. Diagnosis and treatment of hemophagocytic lymphohistiocytosis in children with HLH-2004 protocol[J]. J Clin Pediatr, 2009, 21(8): 221-224.
9. Janka GE, Schneider EM. Modern management of children with haemophagocytic lymphohistiocytosis[J]. Br J Haematol, 2004, 124(1): 4-14.
10. Janka GE. Hemophagocytic syndromes[J]. Blood Rev, 2007, 21(5): 245-253.
11. 薛红漫, 岑丹阳, 夏焱, 等. 噬血细胞综合征-04方案治疗小儿EB病毒相关性噬血细胞综合征[J]. 中华妇幼临床医学杂志, 2009, 5(3): 56-59.
12. Imashulcu S, Kuriyama K, Teramura T, et al. Requirement for Etoposide in the treatment of Epstem-Barr virus associated hemophagocytic lymphohistioeytosis[J]. J clm Onool, 2001, 19(10): 2665-2673.
13. 李欣, 徐酉华. 18例EB病毒相关性噬血淋巴组织细胞增生症的临床分析[J]. 中国小儿血液与肿瘤杂志, 2009, 14(3): 131-133.
14. 朱音, 高怡瑾, 朱晓华, 等. HLH-2004方案诊断治疗小儿噬血细胞综合征结果分析[J]. 临床儿科杂志, 2009, 27(8): 718-722.

West China Medical Journal

Hemophagocytic Lymphohistiocytosis with Erythra as an Outstanding Manifest: A Case Report and the Literature Review

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