CAJ | 학술논문

目的探讨中国人淋巴母细胞淋巴瘤/白血病(LBL)临床病理学特征、临床生物学行为及预后相关因素.方法收集确诊的LBL病例105例,应用HE、免疫组织化学(EnVision二步法),观察临床病理学及免疫表型特点.结果 105例LBL患者,男女比例67:38.发病年龄0～73岁,中位年龄13岁.以淋巴结发病常见(53/105,50.48％),其中颈部淋巴结最为常见(34/53,64.15％)；结外以纵隔最为多见(12/52,23.08％)；69例(69/105,65.71％)出现骨髓累及,15例表现为首发症状.肿瘤细胞表达Ki-67(阳性率5％～90％)；TdT、CD99、CD3(ε)、CD20、CD79a、PAX5、MPO、CD34及CD117的阳性率分别为84.76％(89/105)、85.00％(68/80)、54.37％(56/103)、16.67％(16/96)、40.00％(8/20)、46.67％(28/60)、14.29％(8/56)、25.00％(4/16)及0.根据肿瘤细胞免疫表型标记结果将所有病例分为T细胞型62例(59.05％,62/105),B细胞型33例(31.43％,33/105),非T非B9例(8.57％,9/105),1例(0.95％,1/105)同时表达T、B淋巴细胞标志物.61例检测髓系标志物,8例(13.11％,8/61)有不同程度的表达,其中T细胞型4例、B细胞型4例.105例获随访资料,中位生存期36个月,1、2及3年生存率分别为66.67％(70/105)、48.81％(41/84)及20.69％(12/58).免疫表型、骨髓侵犯以及骨髓移植与预后无明显相关性(P＞0.05).年龄与预后明显相关,儿童和青少年预后明显好于成年人(P=0.032).结论中国人LBL多发于儿童和青少年,男性多见,临床主要表现为多发淋巴结肿大,骨髓累及常见.临床生物学行为为侵袭性,年龄与临床预后相关,儿童和青少年预后明显好于成年人.
목적 탐토중국인림파모세포림파류/백혈병(LBL)림상병이학특정、림상생물학행위급예후상관인소.방법 수집학진적LBL병례105례,응용HE、면역조직화학(EnVision이보법),관찰림상병이학급면역표형특점.결과 105례LBL환자,남녀비례67:38.발병년령0～73세,중위년령13세.이림파결발병상견(53/105,50.48％),기중경부림파결최위상견(34/53,64.15％)；결외이종격최위다견(12/52,23.08％)；69례(69/105,65.71％)출현골수루급,15례표현위수발증상.종류세포표체Ki-67(양성솔5％～90％)；TdT、CD99、CD3(ε)、CD20、CD79a、PAX5、MPO、CD34급CD117적양성솔분별위84.76％(89/105)、85.00％(68/80)、54.37％(56/103)、16.67％(16/96)、40.00％(8/20)、46.67％(28/60)、14.29％(8/56)、25.00％(4/16)급0.근거종류세포면역표형표기결과장소유병례분위T세포형62례(59.05％,62/105),B세포형33례(31.43％,33/105),비T비B9례(8.57％,9/105),1례(0.95％,1/105)동시표체T、B림파세포표지물.61례검측수계표지물,8례(13.11％,8/61)유불동정도적표체,기중T세포형4례、B세포형4례.105례획수방자료,중위생존기36개월,1、2급3년생존솔분별위66.67％(70/105)、48.81％(41/84)급20.69％(12/58).면역표형、골수침범이급골수이식여예후무명현상관성(P＞0.05).년령여예후명현상관,인동화청소년예후명현호우성년인(P=0.032).결론 중국인LBL다발우인동화청소년,남성다견,림상주요표현위다발림파결종대,골수루급상견.림상생물학행위위침습성,년령여림상예후상관,인동화청소년예후명현호우성년인.
Objective To study the clinicopathologic features and prognosis factor of Chinese lymphoblastic lymphoma.Methods 105 LBL cases were collected.Routine HE staining,immunostaining were used to investigate the clinicopathologic features,immunotype.Results The ratio of male to female was 1.76:1 (67:38),the medial age was 13 years old (0-73 years old).53 cases (53/105,50.48 ％) primarily showed lymph node involvement,including 34 cases (34/53,64.15 ％) showed jugular node involvement;mediastinum (12/52,23.08 ％) was the most frequent extranodal involvement site.69 cases (69/105,65.71％)showed bone marrow involvement with 15 cases as the primary involvement.The expression of TdT,CD99,CD3(E),CD20,CD79a,PAX5,MPO,CD34 and CD117 was 84.76 ％ (89/105),85.00 ％ (68/80),54.37 ％ (56/103),16.67 ％ (16/96),40.00 ％ (8/20),46.67 ％ (28/60),14.29 ％ (8/56),25.00 ％ (4/16) and 0.All cases were divided in to two groups,62 cases as T-LBL (59.05 ％,62/105) and 33 cases as B-LBL (31.43 ％,33/105),with 9 cases (8.57 ％,9/105) without the expression of T or B cell marker and 1 cases with the expression of both T and B cell marker.61 cases were detected the expression of myeloid markers and 8 cases were positive.All cases were followed up.The medial survival was 36 months.The survival at 1 year,2 year and 3 year was 66.67 ％ (70/105),48.81％ (41/84) and 20.69 ％ (12/58) respectively.Age was the prognosis associated factor.The children had better progonosis than adults.Immunotype,bone marrow involovement and transplantation didn' t show prognosis indicator (P ＞ 0.05).Conclusion Most of Chinese LBL occurs in child and younger male,multi-lymphadehypertrophy and bone marrow involvement are common.LBL is an aggressive tumor.Age is the prognosis associated factor.Children have a better prognosis than the adult.