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乙肝肝硬化并发皮下脂膜炎样T细胞淋巴瘤伴噬血细胞综合征的治疗分析

杨喜晶,  周福平,  张迁,  钱其军

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文章导航 > 药学实践与服务  > 2015 >  33(4): 363-365
杨喜晶, 周福平, 张迁, 钱其军. 乙肝肝硬化并发皮下脂膜炎样T细胞淋巴瘤伴噬血细胞综合征的治疗分析[J]. 药学实践与服务, 2015, 33(4): 363-365. doi: 10.3969/j.issn.1006-0111.2015.04.019
引用本文: 杨喜晶, 周福平, 张迁, 钱其军. 乙肝肝硬化并发皮下脂膜炎样T细胞淋巴瘤伴噬血细胞综合征的治疗分析[J]. 药学实践与服务, 2015, 33(4): 363-365. doi: 10.3969/j.issn.1006-0111.2015.04.019
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YANG Xijng, ZHOU Fuping, ZHANG Qian, QIAN Qijun. Treatment analysis of liver cirrhosis with hepatitis B complicated by subcutaneous panniculitis-like T-cell lymphoma associated hemophagocytic syndrome[J]. Journal of Pharmaceutical Practice and Service, 2015, 33(4): 363-365. doi: 10.3969/j.issn.1006-0111.2015.04.019
Citation: YANG Xijng, ZHOU Fuping, ZHANG Qian, QIAN Qijun. Treatment analysis of liver cirrhosis with hepatitis B complicated by subcutaneous panniculitis-like T-cell lymphoma associated hemophagocytic syndrome[J]. Journal of Pharmaceutical Practice and Service, 2015, 33(4): 363-365. doi: 10.3969/j.issn.1006-0111.2015.04.019
shu

乙肝肝硬化并发皮下脂膜炎样T细胞淋巴瘤伴噬血细胞综合征的治疗分析

doi: 10.3969/j.issn.1006-0111.2015.04.019

  • 第二军医大学附属东方肝胆外科医院生物治疗科, 上海 200438

Treatment analysis of liver cirrhosis with hepatitis B complicated by subcutaneous panniculitis-like T-cell lymphoma associated hemophagocytic syndrome

  • Department of Bio-therapy, Eastern Hepatobiliary Surgical Hospital affliated to Second Military Medical University, Shanghai 200438, China

  • 摘要: 目的 探讨乙肝肝硬化并发皮下脂膜炎样T细胞淋巴瘤(SPTCL)伴噬血细胞综合征(HPS)的临床特征和治疗方法。 方法 回顾性分析2014年8月第二军医大学附属东方肝胆外科医院1例乙肝肝硬化并发SPTCL伴HPS病例的临床资料。 结果 T细胞受体(TCR)表型不同则该病的侵袭性、治疗反应性及预后明显不同,伴HPS者治疗效果不佳,生存期短。 结论 乙肝肝硬化并发SPTCL伴HPS罕见,早期骨髓形态学、病理学、免疫组化及基因重排检测对确诊有重要意义,及早有效控制乙肝病毒尤为重要。早期诊断及治疗对延长患者生存期有重要意义。
    Abstract: Objective To investigate the clinical features and treatment of liver cirrhosis with hepatitis B complicated by subcutaneous panniculitis-like T-cell lymphoma (SPTCL) associated hemophagocytic syndrome (HPS). Methods A retrospective analysis of case clinical data with liver cirrhosis complicated by SPTCL associated HPS was done in August 2014 in our hospital. Results Because of different phenotypes of T cell receptor (TCR), the aggression, treatment response and prognosis of the disease were significantly different. The patients with HPS had poor treatment effect and short survival period. Conclusion Liver cirrhosis with hepatitis B complicated by SPTCL associated HPS is rare, Bone marrow morphology, pathology, immunohistochemistry and gene rearrangement detection as soon as possible are important for early diagnosis.To control HBV early and effectively is particularly important. Early diagnosis and treatment are important to prolong survival.
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    [2] Go RS, Wester SM.Immunophenotypic and molecular features, clinical outcomes, treatments and prognostic factors associated with subcutaneous panniculitis-like T-cell lymphoma: a systematic analysis of 156 patients reported in the literature[J].Cancer, 2004,l01(6):1404-1413.
    [3] Takeshita M,Okamura S,Oshiro Y, et al.Clinicopathologic differences between 22 cases of CD56-negative and CD56-positive subcutaneous panniculitis-like lymphoma in Japan[J].Hum Pathol,2004,35(2):231-239.
    [4] 马 兰,柴俊月,郑雯琦,等继发于淋巴瘤的嗜血细胞综合征6例临床分析[J].世界最新医学信息文摘,2014,14(2):96.
    [5] Huang JJ, Cai MY, Ye S,et al. Clinical analysis of 19 cases of subcutaneous panniculitis T-cell lymphoma with literature review[J]. Ai Zheng,2009,28 (10): 1093-1099.
    [6] Briki H,Bouaziz J D,Molinier-Frenkel V, et al.Subcutaneous panniculitis-like T-cell lymphoma αβ:complete sustained remission with corticosteroids and methotrexate[J].Br J Dermatol, 2010,163(5):1136-1138.
    [7] Guenova E, Schanz S, Hoetzenecker W, et al. Systemic corticosteroids for subcutaneous panniculitis-like T-cell lymphoma[J]. Br J Dermatol,2014,171 (4): 891-894.
    [8] Sakurai E, Satoh T, Akiko YA, et al. Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) with hemophagocytosis (HPS): successful treatment using high-dose chemotherapy (BFM-NHL & ALL-90) and autologous peripheral blood stem cell transplantation[J]. J Clin Exp Hematop, 2013,53 (2): 135-140.
    [9] Chen R, Liu L,Liang YM,et al. Treatment relapsed subcutaneous panniculitis-like T-cell lymphoma together HPS by cyclosporin A[J]. Hematol Rep,2010,2 (1): e9.
    [10] Zhang X,Schlaak M,Fabri M, et al.Successful treatment of a panniculitis-like primary cutaneous T-Cell lymphoma of the α/β type with bexarotene[J].Case Rep Dennatol,2012,4(1):56-60.
    [11] Bashey S,Krathen M,Abdulla F, et al.Romidepsin is effective in subcutaneous panniculitis-like T-cell lymphoma[J].J Clin Oncol, 2012,30(24):e221-225.
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  • 收稿日期:  2015-02-11
  • 修回日期:  2015-05-14

乙肝肝硬化并发皮下脂膜炎样T细胞淋巴瘤伴噬血细胞综合征的治疗分析

doi: 10.3969/j.issn.1006-0111.2015.04.019
  • 第二军医大学附属东方肝胆外科医院生物治疗科, 上海 200438
  • 收稿日期: 2015-02-11
  • 修回日期: 2015-05-14

摘要: 目的 探讨乙肝肝硬化并发皮下脂膜炎样T细胞淋巴瘤(SPTCL)伴噬血细胞综合征(HPS)的临床特征和治疗方法。 方法 回顾性分析2014年8月第二军医大学附属东方肝胆外科医院1例乙肝肝硬化并发SPTCL伴HPS病例的临床资料。 结果 T细胞受体(TCR)表型不同则该病的侵袭性、治疗反应性及预后明显不同,伴HPS者治疗效果不佳,生存期短。 结论 乙肝肝硬化并发SPTCL伴HPS罕见,早期骨髓形态学、病理学、免疫组化及基因重排检测对确诊有重要意义,及早有效控制乙肝病毒尤为重要。早期诊断及治疗对延长患者生存期有重要意义。

English Abstract

杨喜晶, 周福平, 张迁, 钱其军. 乙肝肝硬化并发皮下脂膜炎样T细胞淋巴瘤伴噬血细胞综合征的治疗分析[J]. 药学实践与服务, 2015, 33(4): 363-365. doi: 10.3969/j.issn.1006-0111.2015.04.019
引用本文: 杨喜晶, 周福平, 张迁, 钱其军. 乙肝肝硬化并发皮下脂膜炎样T细胞淋巴瘤伴噬血细胞综合征的治疗分析[J]. 药学实践与服务, 2015, 33(4): 363-365. doi: 10.3969/j.issn.1006-0111.2015.04.019
shu
YANG Xijng, ZHOU Fuping, ZHANG Qian, QIAN Qijun. Treatment analysis of liver cirrhosis with hepatitis B complicated by subcutaneous panniculitis-like T-cell lymphoma associated hemophagocytic syndrome[J]. Journal of Pharmaceutical Practice and Service, 2015, 33(4): 363-365. doi: 10.3969/j.issn.1006-0111.2015.04.019
Citation: YANG Xijng, ZHOU Fuping, ZHANG Qian, QIAN Qijun. Treatment analysis of liver cirrhosis with hepatitis B complicated by subcutaneous panniculitis-like T-cell lymphoma associated hemophagocytic syndrome[J]. Journal of Pharmaceutical Practice and Service, 2015, 33(4): 363-365. doi: 10.3969/j.issn.1006-0111.2015.04.019
shu

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