肝脾T细胞淋巴瘤临床特征分析(附4例)

Clinical analysis of HSTCL(4 cases)

目的:探讨肝脾T细胞淋巴瘤(hepatosplenic T-cell lymphoma,HSTCL)的临床表现、病理学特征、免疫表型特点。方法:回顾性分析2014年9月-2021年12月我院收治的4例肝脾T细胞淋巴瘤(HSTCL)患者临床资料。结果:4例患者中男性3例,女性1例,诊断时平均年龄为55岁,所有患者均有脾大,其中3例有骨髓受累,4例患者均经脾脏病理活检和/或骨髓免疫分型明确诊断为HSTCL,其中2例为γδ型,1例为αβ型,1例不确定亚型,4例均合并TCR基因重排,1例合并7号染色体异常,4例患者从有临床症状到确诊时间平均时间为10.3月。结论:肝脾T细胞淋巴瘤在老年患者中有一定比例,临床易误诊、漏诊,脾脏病理联合脾脏制备单个核细胞行免疫分型可能提高敏感性,骨髓受累时免疫分型为明确诊断的重要指标;该型淋巴瘤呈侵袭性进展,预后差。

Objective:To investigate the clinical,pathological and immunophenotypic features of hepatosplenic T-cell lymphoma(HSTCL).Methods:From September 2014 to December 2021,the clinical data of 4 patients with hepatosplenic T-cell lymphoma(HSTCL)in our hospital were analyzed retrospectively.Results:Of the 4 patients,3 were male and 1 was female.The average age at diagnosis was 55 years.All patients had splenomegaly,and 3 patients had bone marrow involvement,all of the 4 patients were diagnosed as HSTCL by spleen biopsy and/or bone marrow immunophenotyping.Among them,2 cases wereγδ,1 case wasαβ,1 case was indeterminate subtype,4 cases were complicated with TCR gene rearrangement,one patient was complicated with chromosome 7 abnormality,and the average time from clinical symptoms to diagnosis was 10.3 months.Conclusion:T-cell lymphoma of liver and spleen is one of the most common T-cell lymphomas in elderly patients,and it is easy to be misdiagnosed and missed in clinic,immunophenotype is an important index for definite diagnosis of bone marrow involvement,and this type of lymphoma presents aggressive progression and poor prognosis.