淋巴瘤相關噬血細胞綜合征的研究進展_West China Medical Journal

Authors：

 李娜 ,  鄒立群

四川大學華西醫院腫瘤中心（成都 610041）;

Corresponding?author：

鄒立群, Email: zliqun@hotmail.com

Keywords：

淋巴瘤; 噬血細胞綜合征; 單核-巨噬細胞系統; 細胞因子

DOI：

10.7507/1002-0179.201600399

Video：

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Abstract Full text Figures/Tables Video References Cited by

噬血細胞綜合征（HPS），又稱噬血細胞性淋巴組織細胞增生癥，是一種由多種致病因素導致的單核-巨噬細胞系統反應性增生，釋放大量的細胞因子，引起嚴重器官功能損害的疾病。腫瘤相關性HPS多見于淋巴瘤，又稱淋巴瘤相關噬血細胞綜合征（LAHS）。臨床特征主要有發熱、肝脾腫大、血細胞減少、凝血障礙、高鐵蛋白血癥、高三酰甘油血癥、低纖維蛋白原血癥、骨髓及其他臟器出現噬血細胞現象。LAHS病情兇險，病變進展快，預后差。化學療法聯合大劑量皮質類固醇激素及免疫抑制治療是該病的主要治療方案，但療效差，患者多在短期內死亡。該文就LAHS的發病機制、臨床特征、診斷、治療等方面的研究進展加以綜述，旨在提高對LAHS的認識，指導臨床治療。

Citation： 李娜, 鄒立群. 淋巴瘤相關噬血細胞綜合征的研究進展. West China Medical Journal, 2016, 31(8): 1467-1470. doi: 10.7507/1002-0179.201600399 Copy

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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.
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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.
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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, Vaktn?s 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.	張燕, 洪小南.結外NK/T細胞淋巴瘤嗜血細胞綜合征23例回顧性分析[J].中華腫瘤防治雜志, 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, 賈國存.噬血細胞性淋巴組織細胞增多癥發病機制研究進展[J].中國小兒血液與腫瘤雜志, 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, Vaktn?s 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. 張燕, 洪小南.結外NK/T細胞淋巴瘤嗜血細胞綜合征23例回顧性分析[J].中華腫瘤防治雜志, 2013, 20(21):1672-1675.

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West China Medical Journal

淋巴瘤相關噬血細胞綜合征的研究進展

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