ZAP-70在慢性淋巴细胞白血病/小淋巴细胞淋巴瘤中表达及其与预后的关系

目的 探讨慢性淋巴细胞白血病/小淋巴细胞淋巴瘤(CLL/SLL)病理学特点及ZAP-70蛋白表达的预后意义.方法 采用免疫组织化学EliVision法,回顾性检测ZAP-70在52例CLL/SLL患者淋巴结和骨髓活检组织中的表达特点,并随访患者.结果 (1)病理学特征:12例淋巴结呈现均一性成熟小淋巴细胞增生,6例可见增殖中心.40例骨髓中瘤细胞形态与淋巴结的相似,但未见增殖中心.浸润模式分为结节型(9例)、间质型(3例)、混合型(9例)、弥漫型(19例)4种,比较非弥漫型和弥漫型存活率,二者差异无统计学意义(Fisher精确检验,P=0.199).(2)ZAP-70表达定位在细胞质和细胞核,21例(40.4%)患者ZAP-70阳性,其中因本病或感染等相关因素死亡11例.31例(59.6%)ZAP-70阴性,包括因本病或感染死亡4例.比较二组生存期,阳性组明显较阴性组短(39个月和59个月,x2=6.991,P=0.008).(3)随访51例患者,死亡21例,其中因本病及感染等相关因素死亡15例.结论 CLL/SLL患者ZAP-70阳性表达提示预后不良.

Clinical significance of ZAP-70 protein expression in chronic lymphocytic leukemia/small lymphocytic lymphoma

Objective To study the clinicopathologic features and prognostic significance of ZAP-70 protein expression in chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL). Methods The histologic features of 52 cases of CLL/SLL with lymph node and/or bone marrow biopsies performed were retrospectively reviewed, lmmunohistochemical study using EliVision for ZAP-70 protein was adopted.Results The lymph nodes of the 12 cases studied showed effacement of the nodal architecture and was replaced by a monotonous infiltration of small lymphoid cells. Among them, proliferation centers were identified in 6 cases. Similar morphologic pattern was seen in the 40 bone marrow biopsy samples, but no proliferation center formation obtained. The infiltration pattern of tumor cells in the bone marrow were further subdivided into nodular (n=9), interstitial(n=3), mixed (n=9) and diffuse types (n=19). There was no significant difference found on survival rates between the diffuse infiltration and non-diffuse infiltration groups (Fisher's exact test, P=0. 199). ZAP-70 protein was mainly located in the cytoplasm and nuclei of lymphoma cells. There were 21 cases (40.4%) positive for ZAP-70 and among them, 11 died of this disease or the related infections. On the other hand, ZAP-70 was negative in 31 cases (59. 6% ) and only 4 of them died of this disease or related infections. The overall survival in ZAP-70-negative group was higher than that of the ZAP-70-positive group (59 months versus 39 months, X2=6. 991, P=0. 008). Follow-up information was available in 51 patients. Among the 21 dead cases, 15 died of CLL/SLL or the related infection. Conclusion A positive expression of ZAP-70 protein in CLL/SLL suggests a poor prognosis.