淋巴瘤相关噬血细胞综合征的研究进展_West China Medical Journal

Authors：

 李娜 ,  邹立群

四川大学华西医院肿瘤中心（成都 610041）;

Corresponding author：

邹立群, Email: zliqun@hotmail.com

Keywords：

淋巴瘤; 噬血细胞综合征; 单核-巨噬细胞系统; 细胞因子

DOI：

10.7507/1002-0179.201600399

Video：

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Abstract Full text Figures/Tables Video References Cited by

噬血细胞综合征（HPS），又称噬血细胞性淋巴组织细胞增生症，是一种由多种致病因素导致的单核-巨噬细胞系统反应性增生，释放大量的细胞因子，引起严重器官功能损害的疾病。肿瘤相关性HPS多见于淋巴瘤，又称淋巴瘤相关噬血细胞综合征（LAHS）。临床特征主要有发热、肝脾肿大、血细胞减少、凝血障碍、高铁蛋白血症、高三酰甘油血症、低纤维蛋白原血症、骨髓及其他脏器出现噬血细胞现象。LAHS病情凶险，病变进展快，预后差。化学疗法联合大剂量皮质类固醇激素及免疫抑制治疗是该病的主要治疗方案，但疗效差，患者多在短期内死亡。该文就LAHS的发病机制、临床特征、诊断、治疗等方面的研究进展加以综述，旨在提高对LAHS的认识，指导临床治疗。

Citation： 李娜, 邹立群. 淋巴瘤相关噬血细胞综合征的研究进展. West China Medical Journal, 2016, 31(8): 1467-1470. doi: 10.7507/1002-0179.201600399 Copy

1.	Han AR, Lee HR, Park BB, et al. Lymphoma-associated hemophagocytic syndrome:clinical features and treatment outcome[J]. Ann Hematol, 2007, 86(7):493-498.
2.	Xie W, Hu K, Xu F, et al. Clinical analysis and prognostic significance of lymphoma-associated hemophagocytosis in peripheral T cell lymphoma[J]. Ann Hematol, 2013, 92(4):481-486.
3.	Takahashi N, Miura I, Chubachi A, et al. A clinicopathological study of 20 patients with T/natural killer (NK)-cell lymphoma-associated hemophagocytic syndrome with special reference to nasal and nasal-type NK/T-cell lymphoma[J]. Int J Hematol, 2001, 74(3):303-308.
4.	Henter JI, Horne A, Aricó M, et al. HLH-2004:diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis[J]. Pediatr Blood Cancer, 2007, 48(2):124-131.
5.	Choi YL, Park JH, Kim WS, et al. Aggressive NK-cell leukaemia associated with reactive haemophagocytic syndrome[J]. Clin Exp Dermatol, 2006, 31(1):83-85.
6.	Chuang HC, Lay JD, Hsieh WC, et al. Pathogenesis and mechanism of disease progression from hemophagocytic lymphohistiocytosis to Epstein-Barr virus-associated T-cell lymphoma:nuclear factor-kappa B pathway as a potential therapeutic target[J]. Cancer Sci, 2007, 98(9):1281-1287.
7.	Takai K, Nikkuni K, Sanada M, et al. EB virus-associated peripheral T cell lymphoma presenting with hemophagocytic syndrome and hepatic cell necrosis[J]. Rinsho Ketsueki, 2003, 44(7):462-467.
8.	Han L, Zhang M, Li L, et al. Natural killer/T-cell lymphoma-associated hemophagocytic syndrome:a case report[J]. Oncol Lett, 2014, 8(2):886-890.
9.	任一帆, 贾国存.噬血细胞性淋巴组织细胞增多症发病机制研究进展[J].中国小儿血液与肿瘤杂志, 2014, 19(1):47-50.
10.	Janka GE. Hemophagocytic syndromes[J]. Blood Rev, 2007, 21(5):245-253.
11.	Sano H, Kobayashi R, Tanaka J, et al. Risk factor analysis of non-Hodgkin lymphoma-associated haemophagocytic syndromes:a multicentre study[J]. Br J Haematol, 2014, 165(6):786-792.
12.	Maruoka H, Inoue D, Takiuchi Y, et al. IP-10/CXCL10 and MIG/CXCL9 as novel markers for the diagnosis of lymphoma-associated hemophagocytic syndrome[J]. Ann Hematol, 2014, 93(3):393-401.
13.	Ohno T, Ueda Y, Nagai K, et al. The serum cytokine profiles of lymphoma-associated hemophagocytic syndrome:a comparative analysis of B-cell and T-cell/natural killer cell lymphomas[J]. Int J Hematol, 2003, 77(3):286-294.
14.	Jordan MB, Allen CE, Weitzman SA, et al. How I treat hemophagocytic lymphohistiocytosis[J]. Blood, 2011, 118(15):4041-4052.
15.	Lehmberg K, Mcclain KL, Janka GE, et al. Determination of an appropriate cut-off value for ferritin in the diagnosis of hemophagocytic lymphohistiocytosis[J]. Pediatr Blood Cancer, 2014, 61(11):2101-2103.
16.	Tang Y, Xu X, Song H, et al. Early diagnostic and prognostic significance of a specific Th1/Th2 cytokine pattern in children with haemophagocytic syndrome[J]. Br J Haematol, 2008, 143(1):84-91.
17.	Tsuji T, Hirano T, Yamasaki H, et al. A high sIL-2R/ferritin ration is a useful maker for the diagnosis of lymphoma-associated hemophagocytic syndrome[J]. Ann Hematol, 2014, 93:821-826.
18.	Tabata C, Tabata R. Possible prediction of underlying lymphoma by high sIL-2R/ferritin ratio in hemophagocytic syndrome[J]. Ann Hematol, 2012, 91(1):63-71.
19.	Cho SG, Koh YB, Chang HS, et al. Successful treatment with splenectomy and interferon alpha against recurred hemophagocytic syndrome in remission state of anaplastic large cell lymphoma following high-dose therapy and autologous peripheral blood stem cell transplantation[J]. Eur J Haematol, 2005, 74(3):259-262.
20.	Hoofnagle JH, Dusheiko GM, Schafer DF, et al. Reactivation of chronic hepatitis B virus infection by cancer chemotherapy[J]. Ann Intern Med, 1982, 96(4):447-449.
21.	Li YH, He YF, Jiang WQ, et al. Lamivudine prophylaxis reduces the incidence and severity of hepatitis in hepatitis B virus carriers who receive chemotherapy for lymphoma[J]. Cancer, 2006, 106(6):1320-1325.
22.	Hosing C, Champlin RE. Stem-cell transplantation in T-cell non-Hodgkin's lymphomas[J]. Ann Oncol, 2011, 22(7):1471-1477.
23.	Shimazaki C, Inaba T, Nakagawa M. B-cell lymphoma-associated hemophagocytic syndrome[J]. Leuk Lymphoma, 2000, 38(1/2):121-130.
24.	Machaczka M, Vaktnäs J, Klimkowska M, et al. Malignancy-associated hemophagocytic lymphohistiocytosis in adults:a retrospective population-based analysis from a single center[J]. Leuk Lymphoma, 2011, 52(4):613-619.
25.	Hoffman J, Lossos IS. Lymphoma associated hemophagocytic syndrome:it's going viral[J]. Leuk Lymphoma, 2014, 55(9):1964-1965.
26.	Han L, Li L, Wu J, et al. Clinical features and treatment of natural killer/T cell lymphoma associated with hemophagocytic syndrome:comparison with other T cell lymphoma associated with hemophagocytic syndrome[J]. Leuk Lymphoma, 2014, 55(9):2048-2055.
27.	Yu JT, Wang CY, Yang Y, et al. Lymphoma-associated hemophagocytic lymphohistiocytosis:experience in adults from a single institution[J]. Ann Hematol, 2013, 92(11):1529-1536.
28.	Foss F. Overview of cutaneous T-cell lymphoma:prognostic factors and novel therapeutic approaches[J]. Leuk Lymphoma, 2003, 44(Suppl 3):S55-S61.
29.	Jain S, Zain J, O'connor O. Novel therapeutic agents for cutaneous T-cell lymphoma[J]. I Hematol Oncol, 2012(5):24.
30.	Blom A, Beylot-Barry M, D'incan M, et al. Lymphoma-associated hemophagocytic syndrome (LAHS) in advanced-stage mycosis fungoides/Sézary syndrome cutaneous T-cell lymphoma[J]. J Am Acad Dermatol, 2011, 65(2):404-410.
31.	张燕, 洪小南.结外NK/T细胞淋巴瘤嗜血细胞综合征23例回顾性分析[J].中华肿瘤防治杂志, 2013, 20(21):1672-1675.

1. Han AR, Lee HR, Park BB, et al. Lymphoma-associated hemophagocytic syndrome:clinical features and treatment outcome[J]. Ann Hematol, 2007, 86(7):493-498.
2. Xie W, Hu K, Xu F, et al. Clinical analysis and prognostic significance of lymphoma-associated hemophagocytosis in peripheral T cell lymphoma[J]. Ann Hematol, 2013, 92(4):481-486.
3. Takahashi N, Miura I, Chubachi A, et al. A clinicopathological study of 20 patients with T/natural killer (NK)-cell lymphoma-associated hemophagocytic syndrome with special reference to nasal and nasal-type NK/T-cell lymphoma[J]. Int J Hematol, 2001, 74(3):303-308.
4. Henter JI, Horne A, Aricó M, et al. HLH-2004:diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis[J]. Pediatr Blood Cancer, 2007, 48(2):124-131.
5. Choi YL, Park JH, Kim WS, et al. Aggressive NK-cell leukaemia associated with reactive haemophagocytic syndrome[J]. Clin Exp Dermatol, 2006, 31(1):83-85.
6. Chuang HC, Lay JD, Hsieh WC, et al. Pathogenesis and mechanism of disease progression from hemophagocytic lymphohistiocytosis to Epstein-Barr virus-associated T-cell lymphoma:nuclear factor-kappa B pathway as a potential therapeutic target[J]. Cancer Sci, 2007, 98(9):1281-1287.
7. Takai K, Nikkuni K, Sanada M, et al. EB virus-associated peripheral T cell lymphoma presenting with hemophagocytic syndrome and hepatic cell necrosis[J]. Rinsho Ketsueki, 2003, 44(7):462-467.
8. Han L, Zhang M, Li L, et al. Natural killer/T-cell lymphoma-associated hemophagocytic syndrome:a case report[J]. Oncol Lett, 2014, 8(2):886-890.
9. 任一帆, 贾国存.噬血细胞性淋巴组织细胞增多症发病机制研究进展[J].中国小儿血液与肿瘤杂志, 2014, 19(1):47-50.
10. Janka GE. Hemophagocytic syndromes[J]. Blood Rev, 2007, 21(5):245-253.
11. Sano H, Kobayashi R, Tanaka J, et al. Risk factor analysis of non-Hodgkin lymphoma-associated haemophagocytic syndromes:a multicentre study[J]. Br J Haematol, 2014, 165(6):786-792.
12. Maruoka H, Inoue D, Takiuchi Y, et al. IP-10/CXCL10 and MIG/CXCL9 as novel markers for the diagnosis of lymphoma-associated hemophagocytic syndrome[J]. Ann Hematol, 2014, 93(3):393-401.
13. Ohno T, Ueda Y, Nagai K, et al. The serum cytokine profiles of lymphoma-associated hemophagocytic syndrome:a comparative analysis of B-cell and T-cell/natural killer cell lymphomas[J]. Int J Hematol, 2003, 77(3):286-294.
14. Jordan MB, Allen CE, Weitzman SA, et al. How I treat hemophagocytic lymphohistiocytosis[J]. Blood, 2011, 118(15):4041-4052.
15. Lehmberg K, Mcclain KL, Janka GE, et al. Determination of an appropriate cut-off value for ferritin in the diagnosis of hemophagocytic lymphohistiocytosis[J]. Pediatr Blood Cancer, 2014, 61(11):2101-2103.
16. Tang Y, Xu X, Song H, et al. Early diagnostic and prognostic significance of a specific Th1/Th2 cytokine pattern in children with haemophagocytic syndrome[J]. Br J Haematol, 2008, 143(1):84-91.
17. Tsuji T, Hirano T, Yamasaki H, et al. A high sIL-2R/ferritin ration is a useful maker for the diagnosis of lymphoma-associated hemophagocytic syndrome[J]. Ann Hematol, 2014, 93:821-826.
18. Tabata C, Tabata R. Possible prediction of underlying lymphoma by high sIL-2R/ferritin ratio in hemophagocytic syndrome[J]. Ann Hematol, 2012, 91(1):63-71.
19. Cho SG, Koh YB, Chang HS, et al. Successful treatment with splenectomy and interferon alpha against recurred hemophagocytic syndrome in remission state of anaplastic large cell lymphoma following high-dose therapy and autologous peripheral blood stem cell transplantation[J]. Eur J Haematol, 2005, 74(3):259-262.
20. Hoofnagle JH, Dusheiko GM, Schafer DF, et al. Reactivation of chronic hepatitis B virus infection by cancer chemotherapy[J]. Ann Intern Med, 1982, 96(4):447-449.
21. Li YH, He YF, Jiang WQ, et al. Lamivudine prophylaxis reduces the incidence and severity of hepatitis in hepatitis B virus carriers who receive chemotherapy for lymphoma[J]. Cancer, 2006, 106(6):1320-1325.
22. Hosing C, Champlin RE. Stem-cell transplantation in T-cell non-Hodgkin's lymphomas[J]. Ann Oncol, 2011, 22(7):1471-1477.
23. Shimazaki C, Inaba T, Nakagawa M. B-cell lymphoma-associated hemophagocytic syndrome[J]. Leuk Lymphoma, 2000, 38(1/2):121-130.
24. Machaczka M, Vaktnäs J, Klimkowska M, et al. Malignancy-associated hemophagocytic lymphohistiocytosis in adults:a retrospective population-based analysis from a single center[J]. Leuk Lymphoma, 2011, 52(4):613-619.
25. Hoffman J, Lossos IS. Lymphoma associated hemophagocytic syndrome:it's going viral[J]. Leuk Lymphoma, 2014, 55(9):1964-1965.
26. Han L, Li L, Wu J, et al. Clinical features and treatment of natural killer/T cell lymphoma associated with hemophagocytic syndrome:comparison with other T cell lymphoma associated with hemophagocytic syndrome[J]. Leuk Lymphoma, 2014, 55(9):2048-2055.
27. Yu JT, Wang CY, Yang Y, et al. Lymphoma-associated hemophagocytic lymphohistiocytosis:experience in adults from a single institution[J]. Ann Hematol, 2013, 92(11):1529-1536.
28. Foss F. Overview of cutaneous T-cell lymphoma:prognostic factors and novel therapeutic approaches[J]. Leuk Lymphoma, 2003, 44(Suppl 3):S55-S61.
29. Jain S, Zain J, O'connor O. Novel therapeutic agents for cutaneous T-cell lymphoma[J]. I Hematol Oncol, 2012(5):24.
30. Blom A, Beylot-Barry M, D'incan M, et al. Lymphoma-associated hemophagocytic syndrome (LAHS) in advanced-stage mycosis fungoides/Sézary syndrome cutaneous T-cell lymphoma[J]. J Am Acad Dermatol, 2011, 65(2):404-410.
31. 张燕, 洪小南.结外NK/T细胞淋巴瘤嗜血细胞综合征23例回顾性分析[J].中华肿瘤防治杂志, 2013, 20(21):1672-1675.

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West China Medical Journal

淋巴瘤相关噬血细胞综合征的研究进展

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