成人原发性胃肠道Burkitt样淋巴瘤的诊治

Diagnosis and treatment of primary gastrointestinal Burkitt-like lymphoma in adults

目的:探讨成人原发性胃肠道Burkitt样淋巴瘤的流行病学、临床病理特点、诊断和治疗.方法:利用韩国延世大学图书馆平台在Medline/Pub Med、Web of Science、Directory of Open Access Journals(DOAJ)、Springer Link等15家英文文献数据库和中国知网中文期刊全文数据库中检索1995年以来发表的符合纳入条件的相关文献,共获得来自6篇文献的6例成人原发性胃肠道Burkitt样淋巴瘤的患者资料.结果:成人原发性胃肠道Burkitt样淋巴瘤主要分布在东亚的日本和中国(4例,66.7%);6例患者均为男性,平均年龄57.6岁;2例发生于回盲部,1例发生于胃,1例发生于结肠肝曲,1例发生于末端回肠,还有1例在胃和小肠同时发生;因发现腹部肿块就诊者4例,伴随B症状:体质量减轻4例;乏力2例;所有患者均行消化道内镜检查,行计算机断层扫描(computed tomography,CT)检查者5例,行B超检查者3例;行手术治疗者4例,行联合化疗者5例,行手术+术后化疗者3例;1例患者在1年内死亡;最长1例患者随访5年仍存活;6例患者中行CD20检测者4例,均为阳性;行C D10检测者6例,4例为阳性;行K i-67检测者3例,均为阳性;行B淋巴细胞瘤-2(B-cell lymphoma-2,Bcl-2)检测者3例,均为阴性;行CD23检测者2例,均为阴性;行CD5检测者2例,均为阴性.结论:成人原发性胃肠道Burkitt样淋巴瘤临床罕见,属于高度恶性的非霍奇金淋巴瘤,进展快,死亡率高.其临床表现缺乏特异性,在进行临床干预治疗之前,建议行全面的检查以明确诊断,最终诊断仍依赖于细针穿刺活组织检查或手术切除病理检查.治疗上应在诊断明确后尽早实施外科手术及术后的多药联合辅助化疗.临床医师应该提高对本病的认识,重视该病的临床诊治,以改善患者的预后.

AIM： To investigate the epidemiological and clinicopathological characteristics, diagnosis and treatment of primary gastrointestinal Burkitt-like lymphoma in adults. METHODS： Relevant articles published since 1995 were retrieved in 15 English databases such as MEDLINE/PubMed, Web of Science, Directory of Open Access Journals （DOAJ） and SpringerLink and in China National Knowledge Infrastructure （CNKI） database. Data of six patients with primary gastrointestinal Burkitt-like lymphoma were obtained from six articles.RESULTS： Four cases of adult primary gastrointestinal Burkitt-like lymphoma were from East Asia （Japan and China, 66.7%）. All six patients were male with an average age of 57.6 years. Two cases occurred in the ileocecum, one in the stomach, one in the hepatic flexure of the colon, one in the terminal ileum, and one in the stomach and small intestine simultaneously. Four cases were admitted to the hospital because of abdominal mass, along with B symptoms： weight loss in four cases and fatigue in two cases. All patients underwent alimentary endoscopy （CT scan in 5 cases and B-ultrasound in 3 cases）. Four cases underwent surgery, 5 casesunderwent combined chemotherapy, and 3 cases underwent surgery plus postoperative chemotherapy. One case died within 1 year, and one case was followed for 5 years and is still alive. Immunohistochemically, CD20 and CD10 staining was positive in 4 cases, Ki-67 staining positive in 3 cases, Bcl-2 staining negative in 3 cases, CD23 and CD5 staining negative in 2 cases. CONCLUSION： Adult primary gastrointestinal Burkitt-like lymphoma is a highly malignant non-Hodgkin＇s lymphoma and is extremely rare clinically with rapid development and high mortality. Its clinical manifestations are non-specific, so comprehensive examination should be conducted to confirm the diagnosis before clinical intervention. Final diagnosis still relies on fine-needle aspiration biopsy or surgical excision biopsy. Surgical treatment and multi-drug combination adjuvant chemotherapy postoperatively should be implemented as soon as possible after making a diagnosis. Clinicians should raise their awareness of the disease and pay more attention to the diagnosis and treatment of the disease, which could improve the outcome of the patients.