原发性肝淋巴瘤的临床特点及治疗

Clinical characteristics and treatment of primary hepatic lymphoma

目的探讨原发性肝淋巴瘤（primary hepatic lymphoma，PHL）的临床特征、诊治要点及预后。方法回顾性分析天津市肿瘤医院2005年4月至2010年9月收治的6例PHL患者的临床资料，并结合国内外文献进行分析。结果6例患者中位发病年龄为57岁，男女之比为2：1。首发症状主要为上腹部疼痛、发热。6例均经病理学诊断为非霍奇金淋巴瘤。1例单纯手术治疗（术后失访）。5例术后行CHOP或CHOP样方案化疗，2例健在，2例死亡，1例失访。结论PHL极罕见，临床和影像学表现不典型，易误诊。对肝脏占位性病变伴上腹部不适或发热者，应警惕PHL的可能。其最终确诊依赖组织病理学检查。PHL病理类型主要为非霍奇金淋巴瘤，其中以弥漫大B细胞淋巴瘤最常见。目前尚缺乏标准治疗方法。可选择包括手术、化放疗、生物治疗等在内的综合治疗模式。

Objectives To investigate the clinical features, diagnosis, treatment and prognosis of primary hepatic lymphoma （PHL）. Methods A retrospective study was carried out on the clinical re cords of 6 patients with primary hepatic lymphoma （PHL） treated at the Tianjin Medical University Cancer Hospital from April 2005 to September 2010. The domestic and foreign medical literatures were reviewed. Results For the 6 patients with PHL, the median age was 57 years （range 31-78 years）. The male-to-female ratio was 2 ： 1. The most common initial symptoms were abdominal pain and fever. Serum transaminase, lactate dehydrogenase and [32-microglobulin levels were elevated in 4 of 6 patients. For the 3 patients who were tested for alphafetoprotein and carcinoembryonic antigen levels, the results were normal. None of the patients had a history of hepatitis or cirrhosis. The diagnosis was non-Hodgkin＇s lymphoma. A R0 resection was carried out in 1 patient who was lost to fol-low-up soon after surgery. A R2 resection was carried out in another patient. The remaining 4 patients received liver biopsy. Five patients were treated by CHOP or CHOP-like chemotherapy. A patient died of brain metastases after 8 cycles of chemotherapy, and another patient was lost to follow-up after 1 cycle of chemotherapy. Chemotherapy combined with rituximab were given to the other 3 patients. There was a complete remission in 2 patients after chemotherapy and biotherapy, and these patients were still alive at the last follow-up. Partial remission was achieved in the remaining patient after chemotherapy. The patient was given radiotherapy, but he died finally of tumor progression. Conclusions PHL is an extremely rare lymphoma. Its clinical presentations and imaging manifestations are non-specific. PHL should be considered when the patient has abdominal pain or fever, with a mass in the liver. The ultimate diagnosis depends on histopathologic examination. The pathological type is mainly non Hodgkin＇s lymphoma, with diffuse large B cell lymphoma. There is still no standard treatment. Surgery, chemotherapy, radiotherapy, targeted therapy and biotherapy can be used.