摘要
目的：淋巴母细胞淋巴瘤（LBL）是少见的高度恶性淋巴瘤,WHO新分类法将其归为前体淋巴瘤细胞白血病//淋巴瘤。文中探讨LBL临床和病理学特征。方法：对28例LBL患者的瘤组织做病理、免疫组化检测,并结合临床进行分析。结果：患者中位年龄17（10-42）岁。男26例,女2例。首发症状以颈部淋巴结肿大最多见（32.1%）,其次胸闷、呼吸困难（28.5%）,咳嗽（21.4%）。82.1%患者有纵隔肿大,64.2%有骨髓侵犯。Ⅲ~Ⅳ期21例（76.0%）,T-LBL 26例,B-LBL 2例。TdT和CD99阳性率均为81.2%。平均治疗6个周期,中位生存期9.6个月,Ⅲ~Ⅳ期与Ⅰ~Ⅱ期两组患者生存期无显著性差异（P〉0.05）。1例进行自体造血干细胞移植者获得长生存,2例中枢神经系统复发者均未进行预防。结论：LBL以青少年男性多见,有纵隔占位或伴颈部淋巴结肿大者应考虑LBL可能。LBL以T细胞性为主,TdT阴性不能排除LBL诊断。临床分期与预后未见明显相关。大剂量化疗和造血干细胞移植可能是延长生存期的关键,预防中枢神经系统（CNS）复发很有必要。
Objective：Lymphoblastic lymphoma（LBL） is unusual lymphoma with high malignancy which was classified as precursor lymphocytic leukemia/lymphoma.In this study,the clinicopathological and pathological features（LBL） were investigated.Methods：Clinicopathological study for 28 patients of LBL was reviewed retrospectively from January 2002 to January 2008.Items cover all data concerning clinical manifestations,laboratory investigation and immunohistochemical staining.Results：The patients age ranged from 10 to 42 years,with a median of 17 years,26 patients were male and 2 female.The onset symptom was lymphoadenopathy of neck（32.1%）,chest distress and dyspnea（28.5%） or cough（21.4%）.Bone marrow was involoved in 64.2% patients.76.0% patients were in clinical stages Ⅲ and Ⅳ.26 patients displayed T-cell marker positivity and 2 patients B-cell markers.TdT and CD99 positive reaction both were 81.2%.The 26 patients received average six-cycle chemotherapy.The median survival of the followed-up patients was 9.6 months.The overall surival was no difference between clinical stages Ⅲ-Ⅳ group andⅠ-Ⅱ group.One of 28 patients had long-term survival after autologous stem cell transplantation.2/16 patients without central nervous system（CNS） prophylaxis relapsed in CNS.Conclusion：In adolescent with mediastinal mass and/or lymphoadenopathy of neck,the diagnosis of LBL should be considered,but negative TdT reaction can not exclude this diagnosis.The majority of LBL is T-cell origin.It does not show that clinical stage related with prognosis.High dose chemotherapy and stem cell transplantation may impove suvival,and CNS prophylaxis is necessary.
出处
《医学研究生学报》
CAS
2009年第4期390-393,共4页
Journal of Medical Postgraduates
基金
南京军区总医院重点课题资助项目(批准号:NZ06003)
