合并乙型肝炎感染的肝脾T细胞淋巴瘤：一种罕见的临床病理实体。
Hepatosplenic T-cell lymphoma with coexistent hepatitis B infection: a rare clinicopathologic entity.
作者信息
Siraj Fouzia, Dalal Varsha, Khan Afaq A, Jain Deepali
机构信息
National Institute of Pathology (ICMR), New Delhi - India.
JLNM Hospital, Srinagar, Kashmir - India.
出版信息
Tumori. 2016 Nov 11;102(Suppl. 2):EFAE2F41-1EE6-437B-8640-E3085703C615. doi: 10.5301/tj.5000445.
BACKGROUND
Hepatosplenic T-cell lymphoma (HSTL) is a rare extranodal and systemic lymphoma derived from cytotoxic T cells usually of γδ T cell receptor type. It is characterized by primary extranodal disease with typical sinusoidal infiltration of liver, spleen, and bone marrow by medium-sized lymphoid cells.
CASE REPORT
A 29-year-old man, with no significant prior medical history, presented with fever and massive splenomegaly. A diagnosis of HSTL was established by histologic examination and immunohistochemistry. Staging workup demonstrated bone marrow involvement by lymphoma. In addition, the patient was found to have hepatitis B infection. The association of these 2 entities has been described rarely.
CONCLUSIONS
Hepatosplenic T-cell lymphoma is a distinct T cell lymphoma associated with an aggressive clinical course, a poor response to conventional treatment, and an exceedingly high mortality rate. An association of HSTL with hepatitis B as seen in the present case is exceedingly rare, with few cases reported in the literature.
背景
肝脾T细胞淋巴瘤（HSTL）是一种罕见的结外系统性淋巴瘤，起源于通常为γδT细胞受体类型的细胞毒性T细胞。其特征为原发性结外病变，表现为中等大小淋巴细胞对肝脏、脾脏和骨髓的典型窦状浸润。
病例报告
一名29岁男性，既往无重大病史，出现发热和巨脾。通过组织学检查和免疫组化确诊为HSTL。分期检查显示淋巴瘤累及骨髓。此外，该患者被发现感染乙型肝炎。这两种情况的关联鲜有报道。
结论
肝脾T细胞淋巴瘤是一种独特的T细胞淋巴瘤，临床病程侵袭性强，对传统治疗反应不佳，死亡率极高。如本病例所见，HSTL与乙型肝炎的关联极为罕见，文献中报道的病例很少。
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