摘要
目的探讨套细胞淋巴瘤（MCL）临床病理学特征、分子遗传学异常以及临床生物学行为。方法采用HE、免疫组织化学（EnVision二步法）及间期荧光原位杂交技术（FISH）对114例MCL患者标本进行检查，观察临床病理学及肿瘤细胞免疫表型特点，部分病例标本进行IgH及CCND1基因断裂以及IgH—CCND1融合基因检测。结果114例患者中男女比例为3．56：1，中位年龄60（20—83岁）。发病部位累及淋巴结78例（68．42％），其中颈部49例（62．82％）；累及结外36例（31．58％）。114例患者中累及骨髓23例（20．18％）。肿瘤细胞表达Ki-67（5％-90％），免疫表型CD3s、CD20、CD79a、PAX5、CD5、cyclinDl及Bcl-2的阳性率分别为0％、99．12％、96．43％、97．56％、67．89％、100％及94．12％。FISH检查25例患者中20例（80％）检测到CCND1基因断裂，其中16例（80％）检测到IgH．CCND1融合基因；检查9例患者均检测到IgH基因断裂，其中1例未检测到CCNDl基因断裂及融合基因。75例患者有完整随访资料，随访时间2—57个月，中位生存期40．78个月。1、2及3年生存率分别为84．13％、68．09％及37．50％。Ki-67阳性率〈40％组患者中位生存期较≥40％组明显延长（57个月对36个月，P=0．003）。13例患者接受利妥昔单抗与常规化疗方案联合化疗，7例（53．85％）完全缓解，3例（23．08％）部分缓解，未达中位生存期；44例患者仅接受常规化疗，11例（25．00％）完全缓解，9例（20．45％）部分缓解，中位生存期28个月；两组比较分析P=0．052。结论MCL多发于老年男性，临床主要表现为多发性淋巴结肿大，骨髓累及常见。临床生物学行为为侵袭性，预后较差，Ki-67高表达提示预后不良，利妥昔单抗可明显改善患者生存。肿瘤细胞具有涉及CCND1的特征性基因改变。
Objective To study the clinicopathologic features, immunotype and cytogenetics of Chi- nese mantle cell lymphoma （MCL）. Methods 114 MCL cases were collected from hematopathology lab of department of pathology, Peking University, HSC. Routine HE stain and immune stain were used to investi- gate the clinicopathologic features and immune type. Breaks of CCND1 and IgH/CCND1 fusion genes were detected by FISH. Results The ratio of male to female was 3.56：1 （89：25 ） with the median age of 60 years old （20- 83 years old）. 78 cases （68.42% ,78/114 ） primarily showed lymph node involvement, in- cluding 49 cases（49/78,62.82% ） jugular node involvement; 36 cases （31.58% ,36/114） showed extra- nodal involvement. 23 cases（ 23/114,20.18% ）showed bone marrow involvement. The expressions of CD3a, CD20, CD79a, PAX5, CD5, eyelinD1 and Bcl-2 were 0% （0/114）, 99. 12% （113/114）, 96.43% （27/ 28）, 97.56% （40/41）, 67.89% （74/109）, 100% （114/114） and 94.12% （48/51）, respectively. Break of CCND1 gene was found in 20 cases（80% ,20/25） ,the fusion gene of IgH-CCND1 in 16 cases（80% , 16/ 20） , the break of IgH gene in 9 cases（100% ,9/9） and its fusion gene in 8 cases （88.89% , 8/9）. We fol- lowed up 75 cases with a period of 2 - 57 months. The median survival was 40.78 months. The survivals at 1 year, 2 year and 3 year were 84.13% （53/63） , 68.09% （32/47） and 37.5% （12/32） , respectively. The median survival of group with more than 40% expression of Ki-67 was 36 months, the group with less than 40% expression of Ki67 57 monlhs （ P =0. 003 ）. 7 of 13 patients accepted t）,iltlximalb plus traditional chemo- therapy attained CR, 3 cases PR. 11 of 44 cases ate＇epical traditional chemotllerapy attained CR, 9 cases PR （ P = 0. 052 ）. Conclusion Most of Chinese MCI, or.cured in older male, nmhi-lymphadehypertrophy and bone marrow involvement were common in MCI, as a aggressive tumor. High expression of Ki-67 was an ad- verse prognosfic indicator. Rituximal） couhl improve the survival. Change of CCNDI gene was the most com- mon cytogenetic abnormality.
出处
《中华血液学杂志》
CAS
CSCD
北大核心
2012年第9期738-742,共5页
Chinese Journal of Hematology
基金
北京市自然科学基金（7102101）
