Successful liver transplantation in the treatment of acute liver failure caused by hemophagocytic syndrome: A case report
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关键词:
- 淋巴组织细胞增多症, 嗜血细胞性 /
- 肝功能衰竭, 急性 /
- 肝移植 /
- 治疗学
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表 1 治疗过程中主要化验指标结果
时间点 TBil
(μmol/L)ALT
(U/L)AST
(U/L)PT
(s)INR WBC
(×109/L)Hb
(g/L)PLT
(×109/L)术前 749 83 125 45.9 3.94 2.87 93 98 第4天 94 74 32 12.2 1.05 7.57 88 54 第5天 150 86 72 14.9 1.29 6.42 86 38 第7天 284 2025 4560 22.2 1.93 5.39 81 16 第10天 271 651 432 14.7 1.28 4.31 68 24 第14天 197 43 107 11.1 0.97 11.91 75 125 第28天 41 38 42 11.8 1.04 8.23 96 103 -
[1] RAMOS-CASALS M, BRITO-ZERÓN P, LÓPEZ-GUILLERMO A, et al. Adult haemophagocytic syndrome[J]. Lancet, 2014, 383(9927): 1503-1516. DOI: 10.1016/S0140-6736(13)61048-X. [2] LUO LT, ZHANG X, MA HM, et al. A case of hemophagocytic syndrome with liver failure as the main manifestation[J]. J Clin Hepatol, 2020, 36(2): 412-413. DOI:10.3969/ j.issn. 1001-5256.2020.02.038.罗利婷, 张霞, 马鹤铭, 等. 以肝衰竭为主要表现的噬血细胞综合征1例报告[J]. 临床肝胆病杂志, 2020, 36(2): 412-413. DOI: 10.3969/j.issn.1001-5256.2020.02.038. [3] JAGTAP N, SHARMA M, RAJESH G, et al. Hemophagocytic lymphohistiocytosis masquerading as acute liver failure: A single center experience[J]. J Clin Exp Hepatol, 2017, 7(3): 184-189. DOI: 10.1016/j.jceh.2017.01.119. [4] Chinese Expert Union of Hemophagocytic Syndrome, Hematology Group of Scientific Branch of Chinese Medical Association. Consensus of Chinese experts on the diagnosis and treatment of Hemophilus syndrome[J]. Chin Med J, 2018, 98(2): 91-95. DOI: 10.3760/cma.j.issn.0376-2491.2018.02.004.噬血细胞综合征中国专家联盟, 中华医学会儿科学分会血液学组. 噬血细胞综合征诊治中国专家共识[J]. 中华医学杂志, 2018, 98(2): 91-95. DOI: 10.3760/cma.j.issn.0376-2491.2018.02.004. [5] TAKAHASHI N, CHUBACHI A, KUME M, et al. A clinical analysis of 52 adult patients with hemophagocytic syndrome: The prognostic significance of the underlying diseases[J]. Int J Hematol, 2001, 74(2): 209-213. DOI: 10.1007/BF02982007. [6] CETICA V, SIENI E, PENDE D, et al. Genetic predisposition to hemophagocytic lymphohistiocytosis: Report on 500 patients from the Italian registry[J]. J Allergy Clin Immunol, 2016, 137(1): 188-196. e4. DOI: 10.1016/j.jaci.2015.06.048. [7] CHEN X, WANG F, ZHANG Y, et al. Genetic variant spectrum in 265 Chinese patients with hemophagocytic lymphohistiocytosis: Molecular analyses of PRF1, UNC13D, STX11, STXBP2, SH2D1A, and XIAP[J]. Clin Genet, 2018, 94(2): 200-212. DOI: 10.1111/cge.13363. [8] BRISSE E, WOUTERS CH, ANDREI G, et al. How viruses contribute to the pathogenesis of hemophagocytic lymphohistiocytosis[J]. Front Immunol, 2017, 8: 1102. DOI: 10.3389/fimmu.2017.01102. [9] KARASU Z, KILIC M, CAGIRGAN S, et al. Hemophagocytic syndrome after living-related liver transplantation[J]. Transplant Proc, 2003, 35(4): 1482-1484. DOI: 10.1016/s0041-1345(03)00511-6. [10] SEPULVEDA FE, de SAINT BASILE G. Hemophagocytic syndrome: Primary forms and predisposing conditions[J]. Curr Opin Immunol, 2017, 49: 20-26. DOI: 10.1016/j.coi.2017.08.004. [11] MORIMOTO A, NAKAZAWA Y, ISHⅡ E. Hemophagocytic lymphohistiocytosis: Pathogenesis, diagnosis, and management[J]. Pediatr Int, 2016, 58(9): 817-825. DOI:10.1016/ j.jpeds.2013.06.053. [12] AMMANN S, LEHMBERG K, ZUR STADT U, et al. Primary and secondary hemophagocytic lymphohistiocytosis have different patterns of T-cell activation, differentiation and repertoire[J]. Eur J Immunol, 2017, 47(2): 364-373. DOI: 10.1002/eji.201646686. [13] 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. DOI: 10.1002/pbc.21039. [14] KOU JT, LIU Z, ZHU JQ, et al. Research on clinical efficacy of liver transplantation for severe liver disease[J]. Organ Transpl, 2020, 11(4): 482-486. DOI: 10.3969/j.issn.1674-7445.2020.04.010.寇建涛, 刘喆, 朱继巧, 等. 肝移植治疗危重症肝病的临床疗效研究[J]. 器官移植, 2020, 11(4): 482-486. DOI: 10.3969/j.issn.1674-7445.2020.04.010. [15] FU BS, YI SH, YI HM, et al. Clinical analysis of hemophagocytic syndrome after liver transplantation[J]. J Organ Transplantation, 2013, 4(2): 68-74. DOI:10.3969/ j.issn.1674-7445.2013.02.002.傅斌生, 易述红, 易慧敏, 等. 肝移植术后噬血细胞综合征临床分析[J]. 器官移植, 2013, 4(2): 68-74. DOI: 10.3969/j.issn.1674-7445.2013.02.002. [16] AMIR AZ, LING SC, NAQVI A, et al. Liver transplantation for children with acute liver failure associated with secondary hemophagocytic lymphohistiocytosis[J]. Liver Transplantation, 2016, 22(9): 1245-1253. DOI:10.1016/ S0168-8278(16)00374-3. [17] JANKA GE. Familial and acquired hemophagocytic lymphohistiocytosis[J]. Annu Rev Med, 2012, 63: 233-246. DOI: 10.1146/annurev-med-041610-134208. [18] MATTHES-MARTIN S, PETERS C, FRITSCH G, et al. Successful stem cell transplantation following orthotopic liver transplantation from the same haploidentical family donor in a girl with hemophagocytic lymphohistiocytosis[J]. Blood, 2000, 96(12): 3997-3999. DOI:10.1182/ blood.V96.12.3997.