摘要
目的:报道5例原发肾上腺弥漫大B细胞淋巴瘤(DLBCL)的临床以及病理特点,提高对原发肾上腺DLBCL的认识及诊治水平。方法:回顾性分析在我院诊治的5例原发肾上腺DLBCL患者的临床及病理资料。结果:5例原发肾上腺DLBCL,其中4例为老年男性;临床多以腰痛或腹痛为首发表现,5例患者均伴有B症状,4例伴有乳酸脱氢酶升高,IPI积分多为中高危;3例患者伴有肾上腺皮质功能不全,其中2例需要糖皮质激素替代治疗;影像学均为双侧肾上腺肿块,超声或CT引导下穿刺病理确诊;病理4例呈现双表达DLBCL,活化B细胞来源;未见骨髓受累患者。原发肾上腺DLBCL采用包含利妥昔单抗的联合化疗疗效较理想,但停药易复发,应用来那度胺或沙利度胺维持治疗可能可以减少复发;治疗需要兼顾中枢神经系统淋巴瘤的预防,包括大剂量甲氨蝶呤或来那度胺预防。结论:本研究结果提示发现双侧肾上腺占位需要警惕原发肾上腺淋巴瘤,确诊需要活检病理证实;原发肾上腺DLBCL治疗建议含有利妥昔单抗的联合化疗,同时给予中枢淋巴瘤预防,联合化疗结束后建议维持治疗1~2年有助于减少复发。
Objective:To report the clinical and pathological features of 5 cases of primary adrenal diffuse large B-cell lymphoma(DLBCL),in order to improve the treatment of primary adrenal DLBCL.Method:We reported the clinical and pathological data of 5 patients with primary adrenal DLBCL diagnosed in our hospital.Result:There were 4 elderly men in the 5 patients.The most common primary clinical manifestations were abdominal pain or low back pain.All the 5 cases were with B symptoms.Four of these cases associated with elevated lactate dehydrogenase.Most of these cases are integral to high risk of IPI score.Three of them present with adrenal insufficiency.Two patients require glucocorticoid hormone replacement therapy.All the 5 patients were bilateral adrenal masses.The diagnosis was made by ultrasound or CT-guided biopsy and pathology.Four patients were double expression DLBCL,activating B cell original.There was no patient with bone marrow involved.The treatment of primary adrenal DLBCL with combined chemotherapy containing rituximab was ideal,but it was easy to relapse after stop therapy.Lenalidomide or thalidomide could be used as maintenance therapy to reduce recurrence.Treatment of primary adrenal DLBCL also need to give the prevention of secondary central nervous system lymphoma,including large doses methotrexate or lenalidomide.Conclusion:It suggests that bilateral adrenal placeholder needs to be alert to primary adrenal lymphoma.The diagnosis of primary adrenal DLBCL needs biopsy and pathology confirmation.Recommending treatment of primary adrenal DLBCL is combined chemotherapy containing rituximab.The prevention of central lymphoma and maintenance treatment may help reduce recurrence.
作者
冯茹
王婷
李江涛
张劲松
张野坪
张春丽
杨亚姿
白洁菲
刘辉
FENG Ru;WANG Ting;LI Jiangtao;ZHANG Jinsong;ZHANG Yeping;ZHANG Chunli;YANG Yazi;BAI Jiefei;LIU Hui(Department of Hematology,Beijing Hospital,National Geriatrics Center,Beijing,100730,China)
出处
《临床血液学杂志》
CAS
2019年第6期869-873,共5页
Journal of Clinical Hematology
基金
国家自然科学基金青年项目(No:81600132)
首都临床特色应用研究与成果推广项目(No:Z171100001017200)
关键词
原发肾上腺淋巴瘤
弥漫大B细胞淋巴瘤
临床
病理
primary adrenal lymphoma
diffuse large B-cell lymphoma
clinical
pathology ~*
