儿童噬血细胞综合征的流行病学是怎样的？

2015-06-16 10:32:56 浏览次数：

HPS为罕见病，其发病率目前尚无确切数据。在瑞典、意大利、美国等国家中，成人HPS的年发病率约为1/8000000，儿童HPS的年发病率为1-10/10000000。Ishii等[1]报道日本的HPS年发生率为1/800000。Niece等[2]报道美国德克萨斯州儿童HPS横断面发病率为1.07/100000。血液肿瘤是继发性HPS的常见病因， Machaczka1等[3]报道肿瘤相关性HPS发病率为0.36/100000。其他病因引起的HPS主要发生于儿童，而血液肿瘤相关的HPS主要发生于成人，在儿童HPS患者中相对较少，随着年龄增长，血液肿瘤相关性HPS比例逐渐增高。Yang等[4]报道92例儿童HPS患者中仅6.5%(6/92)为肿瘤相关性HPS。Tseng等[5]报道96例成人HPS患者中63.5%（61/96）为血液肿瘤相关性HPS。另有文献报道[3, 6]，在血液肿瘤相关疾病中，合并HPS的发生率为0.708-0.9%。与HPS相关的常见血液肿瘤主要是淋巴瘤/白血病，而急性淋巴细胞性白血病、急性髓性白血病、骨髓增生异常综合征也可见散在报道[1, 7, 8]。据以往文献报道[9]，淋巴瘤相关性噬血细胞综合征（LAHS）主要发生在亚洲人群，以外周T/NK细胞淋巴瘤为主，少数为B细胞淋巴瘤。而西方国家相对少见，两种类型的淋巴瘤发生率大致均等。
各个国家的HPS的发病率及病因不一样，参见图1.
参考文献：
[1] Ishii, E., S. Ohga, S. Imashuku, et al., Nationwide survey of hemophagocytic lymphohistiocytosis in Japan. Int J Hematol, 2007. 86(1): p. 58-65.
[2] Niece, J.A., Z.R. Rogers, N. Ahmad, et al., Hemophagocytic lymphohistiocytosis in Texas: observations on ethnicity and race. Pediatr Blood Cancer, 2010. 54(3): p. 424-8.
[3] Machaczka, M., J. Vaktnas, M. Klimkowska, et al., Malignancy-associated hemophagocytic lymphohistiocytosis in adults: a retrospective population-based analysis from a single center. Leuk Lymphoma, 2011. 52(4): p. 613-9.
[4] Yang, S., L. Zhang, C. Jia, et al., Frequency and development of CNS involvement in Chinese children with hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer, 2010. 54(3): p. 408-15.
[5] Tseng, Y.T., W.H. Sheng, B.H. Lin, et al., Causes, clinical symptoms, and outcomes of infectious diseases associated with hemophagocytic lymphohistiocytosis in Taiwanese adults. J Microbiol Immunol Infect, 2011. 44(3): p. 191-7.
[6] Majluf-Cruz, A., R. Sosa-Camas, O. Perez-Ramirez, et al., Hemophagocytic syndrome associated with hematological neoplasias. Leuk Res, 1998. 22(10): p. 893-8.
[7] Jordan, M.B., C.E. Allen, S. Weitzman, et al., How I treat hemophagocytic lymphohistiocytosis. Blood, 2011. 118(15): p. 4041-52.
[8] O'Brien, M.M., Y. Lee-Kim, T.I. George, et al., Precursor B-cell acute lymphoblastic leukemia presenting with hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer, 2008. 50(2): p. 381-3.
[9] Allory, Y., D. Challine, C. Haioun, et al., Bone marrow involvement in lymphomas with hemophagocytic syndrome at presentation: a clinicopathologic study of 11 patients in a Western institution. Am J Surg Pathol, 2001. 25(7): p. 865-74.

图1：各国噬血细胞综合征的发病率及病因组成示意图。

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