摘要
目的探讨T淋巴母细胞白血病/淋巴瘤(T-LBL)的病理及临床特征。方法对27例确诊为T-LBL患者的瘤组织行病理组织学、免疫组化分析,并对临床症状特点、诊断、治疗、疗效及生存情况进行分析。结果首发症状以淋巴结肿大多见(81.5%),乏力(63.0%)、咳嗽(48.1%)次之。70.4%有纵隔包块,29.6%存在多浆膜腔积液,70.4%骨髓浸润,3.7%出现皮肤、口腔黏膜广泛浸润。Ⅲ～Ⅳ期25例(92.6%),Ⅱ期2例(7.4%)。TdT、CD3及CD99的阳性率分别为100.0%、81.5%及85.2%,CD43、CD2、CD4、CD7、CD8有不同程度表达。平均治疗5个疗程,中位生存时间11个月,存在多浆膜腔积液的患者生存时间均小于6个月。3例行造血干细胞移植及1例予大剂量化疗患者已获得2年以上生存。3例出现中枢神经系统浸润及1例出现皮肤、口腔黏膜广泛浸润者仅生存1.5个月。结论 T-LBL是高度恶性淋巴瘤,预后极差,造血干细胞移植是治疗本病、延长患者生存期的一种有效的方法。
Objective To explore the pathological and clinical characteristic of T lymphoblastic leukemia（T-LBL） /lymphoma.Methods Tumor tissue from 27 patients with confirmed diagnosis of T-LBL were subjected to histopathological and immunohistochemical analysis,and their clinical symptom features,diagnosis,treatment,curative effects and survival conditions were analyzed.Results The most common very early signs and symptoms were lymphadenectasis（81.5%）,followed by fatigue（63.0%） and cough（48.1%）.There were 70.4% of patients with mediastinal mass,29.6% with multiple serous cavity effusion,70.4% with bone marrow invasion and 3.7% with extensive invasion of skin and oral mucosa.25 cases（92.6%） were in phase Ⅲ-Ⅳ,and 2 cases（7.4%） in phaseⅡ.Positive rate of terminal deoxynucleotidyl transferase（TdT）,CD3 and CD99 were 100.0%,81.5% and 85.2%,respectively,and different degrees of CD43,CD2,CD4,CD7 and CD8 expression were demonstrated.After an average of 5 courses of treatment,the median survival time of patients was 11 months,and the survival time of patients with multiple serous cavity effusion was all less than 6 months.More than 2 years survival had been found in 3 cases of hematopoietic stem cell transplantation and one case of high-dose chemotherapy.3 patients with tumor invasion in central nervous system and one patient with skin and oral mucosa extensive invasion survived only 1.5 months.Conclusion Hematopoietic stem cell transplantation is an effective approach to treat T-LBL,a highly malignant lymphoma with very poor prognosis,and prolong survival.
出处
《重庆医学》
CAS
CSCD
北大核心
2011年第34期3447-3449,I0002,共4页
Chongqing medicine
基金
第三军医大学第一附属医院临床创新基金资助项目(SWH2006B007)
关键词
淋巴瘤
T细胞
病理状态
体征和症状
免疫组织化学
造血干细胞移植
预后
lymphoma
T-cell
pathological conditions
signs and symptoms
immunohistochemistry
hematopoietic stem cell transplantation
prognosis
