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原发性纵隔大B细胞淋巴瘤(PMBL)是一种非霍奇金淋巴瘤的独特亚型,好发于青年女性,起源于胸腺髓质B细胞,主要表现为前上纵隔巨大肿块,患者症状与肿瘤的胸腔内浸润和压迫邻近器官有关。病理学表现为中等到大体积的肿瘤细胞弥漫增生,肿瘤细胞周围常有不同程度的纤维化和(或)硬化。PMBL表达多重成熟B细胞抗原和B细胞转录因子,但存在缺陷的免疫球蛋白产生。细胞遗传学涉及JAK2基因和c-REL基因的扩增。由于缺乏前瞻性的随机临床试验,目前尚未见PMBL患者治疗的最佳方案。推荐以蒽环类药物为基础的方案化学治疗(化疗),化疗结束后正电子发射计算机断层显像-CT评估是否辅助放射治疗(放疗)。推荐使用剂量调整后的依托泊苷+多柔比星+环磷酰胺+长春新碱+泼尼松+利妥昔单抗(DA-EPOCH-R)方案或利妥昔单抗+环磷酰胺+多柔比星+长春新碱+泼尼松+异环磷酰胺+卡铂+依托泊苷(RCHOP-ICE)方案化疗,可减少巩固放疗及其带来的远期副反应。患者预后与2个或以上的结外病变以及所接受的初始治疗关系密切。现就PMBL的最新治疗进展加以综述。
Primary mediastinal large B cell lymphoma (PMBL) is a unique subtype of non-Hodgkin’s lymphoma, which occurs in young women, originating in thymus medulla B cells, mainly in the anterior superior mediastinum huge mass, patients Symptoms and tumor chest infiltration and compression of adjacent organs. Pathological manifestations of medium to large volume of tumor cells diffuse proliferation, tumor cells often have varying degrees of fibrosis and (or) sclerosis around. PMBL expresses multiple mature B cell antigens and B cell transcription factors, but has defective immunoglobulin production. Cytogenetics involves the amplification of the JAK2 and c-REL genes. Due to the lack of prospective randomized clinical trials, no optimal treatment for PMBL patients has been found. Anthracycline-based regimen chemotherapy (chemotherapy) is recommended. Positron emission computed tomography-CT after adjuvant chemotherapy evaluates adjuvant radiation therapy (radiotherapy). Etoposide + doxorubicin + cyclophosphamide + vincristine + prednisone + rituximab (DA-EPOCH-R) regimen or rituximab + cyclophosphamide + Doxorubicin + vincristine + prednisone + ifosfamide + carboplatin + etoposide (RCHOP-ICE) regimen chemotherapy can reduce the consolidation of radiation therapy and its long-term side effects. The patient’s prognosis is closely related to 2 or more extranodal diseases and the initial treatment received. The latest treatment progress of PMBL is reviewed.