淋巴瘤白血病的病理类型与临床特征分析

Analysis of pathological type and clinical features of lymphoma cell leukemia

目的探讨淋巴瘤白血病（LML）患者的病理类型和临床表现。方法按照2008年世界卫生组织（WHO）造血与淋巴组织肿瘤分类方案，回顾性分析692例非霍奇金淋巴瘤（NHL）患者中127例LML患者病理类型与临床特征。应用组织形态学、免疫组织化学、流式细胞术和骨髓检查结合临床资料进行研究。结果15种NHL病理类型发生LML，其中发病率较高的NHL病理类型依次为B原始淋巴细胞淋巴瘤（B—LBL）、小淋巴细胞淋巴瘤（SLL）和T原始淋巴细胞淋巴瘤（T-LBL）。LML的T和B细胞亚型分别为45例和74例。两组总体生存（OS）率差异有统计学意义（P〈0．01）。81例患者初诊时已为LML，46例患者在病程1～88个月发展为LML，原发淋巴结内和淋巴结外者分别为96例和31例，LBL和急性淋巴细胞白血病、SLL和慢性淋巴细胞白血病有不同的发病形式和发展阶段。结论LML并不少见。发生LML的病理类型有15种之多。其临床表现比较特殊，尤其是原发淋巴结外的LML更为独特。

Objective To analyze the pathological type and clinical features of patients with lymphoma cell leukemia （LML）. Methods According to the 2008 WHO classification of tumors of hematopoietic and lymphoid tissue, the pathological type and clinical features of 127 LML cases were analyzed retrospectively. Results There were 15 kinds of NHL developed LML. The incidence in frequent order of them was B-lymphoblastic lymphoma, CLL/small lymphocytic lymphoma （SLL） and T-lymphoblastie lymphoma. The LML of T and B cell subtypes were 45 and 74, respectively. There was a significant difference in overall sur- vival between T-LML and B-LML（P 〈0.01 ）. Eighty one patients presented LML at the same time of the NHL diagnosis and 46 during the course （1 -88 months） of disease. Primary nodal and extranodal NHLs developed LML were 96 and 31 cases, respectively. The clinical manifestations of LBL and SLL patients differed from that of ALL and CLL patients. Conclusion LML is not a rare manifestation of NHL. Pathological types of NHL developed LML are 15 kinds in our patients. The clinical features of LML patients are somewhat special, especially for primary extranodal LML patients.