Liu En-bin, Chen Hui-shu, Zhang Pei-hong, Li Zhan-qi, Sun Qi, Yang Qing-ying, Fang Li-huan, Sun Fu-jun
Department of Pathology, Chinese Academy of Medical Sciences, Tianjin, China.
Zhonghua Bing Li Xue Za Zhi. 2011 Dec;40(12):810-4.
To study the clinicopathologic features of aggressive natural killer cell leukemia (ANKL).
The clinical and pathologic features were analyzed in 10 patients with ANKL. The complete blood count, peripheral blood smears, bone marrow aspirates and bone marrow biopsies were studied. Immunophenotypic analysis was carried out by flow cytometry and immunohistochemistry. T-cell receptor (TCR) γ gene rearrangement was studied by PCR method.
The most frequent hematologic abnormalities observed were anemia (7 cases) and thrombocytopenia (9 cases). Large granular lymphocytes were found on peripheral blood smears of 6 patients. In bone marrow aspirates, lymphocytosis (> 20.0%) was demonstrated in 8 cases and large granular lymphocytes in 6 cases. Bone marrow biopsies revealed various degrees of neoplastic infiltration, as follows: mild (5 cases), moderate (3 cases) and severe (2 cases). The neoplastic cells were mainly interstitial in distribution in 8 cases and diffuse in 2 cases. Hemophagocytosis was observed in 4 cases. Flow cytometry showed CD2+ sCD3- CD4- CD56+ CD57- in all cases, CD7+ in 9 cases, CD16+ in 5 cases, CD8+ in 4 cases and CD5+ in 1 case. Immunohistochemistry performed in 8 cases showed the following results: cCD3+ in 4 cases, CD56+ in 6 cases, TIA-1+ in 6 cases, granzyme B+ in 4 cases and perforin+ in 2 cases. PCR study revealed germline TCRγ gene configuration in all cases.
ANKL is a highly aggressive NK cell-derived lymphoid neoplasm. Comprehensive morphologic, immunophenotypic and molecular analysis are essential in arriving at a correct diagnosis. ANKL needs to be distinguished from other types of NK-cell and T-cell lymphomas.
研究侵袭性自然杀伤细胞白血病（ANKL）的临床病理特征。
对10例ANKL患者的临床和病理特征进行分析。研究全血细胞计数、外周血涂片、骨髓穿刺及骨髓活检结果。采用流式细胞术和免疫组织化学进行免疫表型分析。通过聚合酶链反应（PCR）方法研究T细胞受体（TCR）γ基因重排。
观察到的最常见血液学异常为贫血（7例）和血小板减少（9例）。6例患者外周血涂片发现大颗粒淋巴细胞。骨髓穿刺显示8例淋巴细胞增多（>20.0%），6例有大颗粒淋巴细胞。骨髓活检显示不同程度的肿瘤浸润，如下：轻度（5例）、中度（3例）和重度（2例）。肿瘤细胞分布主要为间质型8例，弥漫型2例。4例观察到噬血细胞现象。流式细胞术显示所有病例均为CD2 + sCD3 - CD4 - CD56 + CD57 - ，9例CD7 + ，5例CD16 + ，4例CD8 + ，1例CD5 + 。8例进行免疫组织化学检测结果如下：4例cCD3 + ，6例CD56 + ，6例TIA - 1 + ，4例颗粒酶B + ，2例穿孔素 + 。PCR研究显示所有病例TCRγ基因构型为种系型。
ANKL是一种高度侵袭性的自然杀伤细胞来源的淋巴瘤。综合形态学、免疫表型和分子分析对于正确诊断至关重要。ANKL需要与其他类型的自然杀伤细胞和T细胞淋巴瘤相鉴别。
