Shi Yang, Wang Endi
From the Department of Pathology, Duke University Medical Center, Durham, North Carolina.
Arch Pathol Lab Med. 2015 Sep;139(9):1173-80. doi: 10.5858/arpa.2014-0079-RS.
Hepatosplenic T-cell lymphoma is a rare, aggressive T-cell lymphoma, characterized by hepatosplenic sinusoidal infiltration of monotonous, medium-sized, nonactivated cytotoxic T cells, usually of γ/δ T-cell receptor type. Hepatosplenic T-cell lymphoma occurs more frequently in immunocompromised patients, especially in those receiving long-term immunosuppressive therapy. Patients usually manifest hepatosplenomegaly without lymphadenopathy. The bone marrow is also involved in two-thirds of cases and is often accompanied by circulating lymphoma cells, which, along with anemia and thrombocytopenia, may raise suspicion for acute leukemia. The differential diagnosis includes aggressive natural killer-cell leukemia, T-large granular lymphocytic leukemia, T-lymphoblastic leukemia, enteropathy-associated T-cell lymphoma type II, primary cutaneous γ/δ T-cell lymphoma, other peripheral T-cell lymphomas, myelodysplastic syndrome, and infectious mononucleosis. The diagnosis is usually established from the combination of clinical findings, histologic features, and immunophenotype, although cytogenetic/molecular studies are occasionally needed. Hepatosplenic T-cell lymphoma exhibits a dismal clinical course with a poor response to currently available therapies.
肝脾T细胞淋巴瘤是一种罕见的侵袭性T细胞淋巴瘤，其特征是肝脾窦内有单一的中等大小、未活化的细胞毒性T细胞浸润，通常为γ/δ T细胞受体类型。肝脾T细胞淋巴瘤在免疫功能低下的患者中更常见，尤其是那些接受长期免疫抑制治疗的患者。患者通常表现为肝脾肿大，无淋巴结病。三分之二的病例骨髓也受累，且常伴有循环淋巴瘤细胞，这与贫血和血小板减少一起，可能会引发对急性白血病的怀疑。鉴别诊断包括侵袭性自然杀伤细胞白血病、T大颗粒淋巴细胞白血病、T淋巴母细胞白血病、II型肠病相关T细胞淋巴瘤、原发性皮肤γ/δ T细胞淋巴瘤、其他外周T细胞淋巴瘤、骨髓增生异常综合征和传染性单核细胞增多症。诊断通常根据临床发现、组织学特征和免疫表型综合确定，尽管偶尔需要细胞遗传学/分子研究。肝脾T细胞淋巴瘤临床病程不佳，对目前可用的治疗反应较差。
