Division of Gastroenterology, Department of Medicine, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, Massachusetts, USA;
J Gastrointest Oncol. 2013 Jun;4(2):131-6. doi: 10.3978/j.issn.2078-6891.2013.017.
A 59-year-old man with diabetes mellitus, prior hepatitis B infection and recently diagnosed cirrhosis with prior Babesiosis presented to our institution from an outside hospital with six months of worsening abdominal pain, myalgias and fevers. On admission, physical examination revealed jaundice, hepatosplenomegaly and diffuse lymphadenopathy. Laboratory investigations demonstrated mild anemia, thrombocytopenia, hyperbilirubinemia and elevated lactate dehydrogenase. Tests for human immunodeficiency virus, and active Babesia microti infection were negative, however Epstein-Barr virus DNA by quantitative PCR was markedly elevated. CT scan revealed features suggestive of a cirrhotic liver without focal mass lesions as well as massive splenomegaly with axillary, retroperitoneal and inguinal lymphadenopathy. Bone marrow and lymph node biopsies were obtained which ultimately revealed hepatosplenic T-cell lymphoma. The patient's initial liver biopsy from five months prior to presentation was re-evaluated by our institution's pathologists. Histologic analysis showed hepatic sinusoidal and portal infiltration of atypical lymphocytes morphologically identical to those present on the more recently excised lymph node tissue. The hepatic sinusoidal lymphoid cells were strongly positive for CD2, CD3 and CD5 whereas CD4, CD8 stained only minor subsets of the T cells. Subsequent flow cytometric immunophenotypying of peripheral blood identified T-cell receptor alpha/beta positive cells that lacked CD4 and CD8 (double negative alpha/beta T cells). Given the established bone marrow involvement, he was diagnosed with stage IV disease and treated with chemotherapy. His clinical course involved multiple hospitalizations complicated by hyponatremia, neutropenic fevers and pulmonary emboli. Following his fourth cycle of chemotherapy, he developed worsening liver failure and expired approximately three months after initial diagnosis of lymphoma. Hepatosplenic lymphoma of alpha/beta T cells is a rare malignancy with largely unclear risk factors and varied clinical presentations. Notably, diffuse infiltration of liver parenchyma is a prominent feature and the disease can mimic cirrhosis clinically as well as radiographically. Early recognition of this aggressive lymphoma is important and should be considered in the evaluation of patients in whom the etiology of cirrhosis remains in question.
一位 59 岁的男性，患有糖尿病、既往乙型肝炎感染和近期诊断的肝硬化，曾因巴贝斯虫病在我院外的一家医院就诊，其病史为六个月的腹痛加重、肌肉疼痛和发热。入院时，体格检查发现黄疸、肝脾肿大和全身淋巴结肿大。实验室检查显示轻度贫血、血小板减少、高胆红素血症和乳酸脱氢酶升高。人类免疫缺陷病毒检测和活动性微小巴贝斯虫感染均为阴性，然而，聚合酶链反应检测到的 Epstein-Barr 病毒 DNA 明显升高。CT 扫描显示肝硬化肝脏的特征，无局灶性肿块病变，以及巨脾伴腋窝、腹膜后和腹股沟淋巴结肿大。进行了骨髓和淋巴结活检，最终发现肝脾 T 细胞淋巴瘤。患者在就诊前五个月的初始肝脏活检由我院病理学家重新评估。组织学分析显示，肝窦和门脉有非典型淋巴细胞浸润，形态上与最近切除的淋巴结组织中的淋巴细胞相同。肝窦内淋巴细胞对 CD2、CD3 和 CD5 呈强阳性，而 CD4、CD8 仅染色 T 细胞的少数亚群。随后对外周血进行流式细胞术免疫表型分析，发现 T 细胞受体 alpha/beta 阳性细胞缺乏 CD4 和 CD8（双阴性 alpha/beta T 细胞）。鉴于已确定的骨髓受累，他被诊断为 IV 期疾病，并接受了化疗。他的临床病程包括多次住院，并发低钠血症、中性粒细胞减少性发热和肺栓塞。在接受第四次化疗后，他出现肝功能衰竭恶化，并在最初诊断为淋巴瘤后约三个月去世。alpha/beta T 细胞肝脾淋巴瘤是一种罕见的恶性肿瘤，其主要危险因素和临床表现各不相同。值得注意的是，肝实质弥漫性浸润是一个突出的特征，该疾病在临床上和影像学上均可模仿肝硬化。早期识别这种侵袭性淋巴瘤很重要，对于那些肝硬化病因仍有疑问的患者，应考虑进行评估。
