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

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。

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

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.