Zhonghua Xue Ye Xue Za Zhi. 2006 Feb;27(2):116-9.
To improve the diagnostic accuracy of aggressive NK-cell leukemia (ANKL).
Retrospective clinical cases were analysed.
The ANKL patients often present with fever, hepatosplenomegaly, jaundice, abnormal liver function and pancytopenia. The disease had an aggressive clinical course. Multiorgan failure and hemophagocytic syndrome were frequent complications. Median survival time was less than 2 months. The diagnostic criteria should be (1) Patients present with fever, hepatosplenomegaly and lymphadenopathy; (2) Neutropenia, anemia and thrombocytopenia with high number of circulating large granular lymphocytes; (3) large granular lymphocytes infiltrated in bone marrow aspirate and core biopsy; (4) immunophenotype: CD2(+), surface CD3(-), cytoplasm CD3(+), CD56(+), CD57(-). CD11b(+/-) and CD16(+/-). No T-cell receptor (TCR) genes rearrangement; (5) EB virus antibody usually positive; (6) No unique karyotypic abnormality, sometimes del (6) (q21q25); (7) Exclusion of other diseases with large granular lymphocytosis.
提高侵袭性自然杀伤细胞白血病（ANKL）的诊断准确性。
对回顾性临床病例进行分析。
ANKL患者常表现为发热、肝脾肿大、黄疸、肝功能异常和全血细胞减少。该病临床病程进展迅速。多器官功能衰竭和噬血细胞综合征是常见并发症。中位生存时间小于2个月。诊断标准应为：（1）患者出现发热、肝脾肿大和淋巴结病；（2）中性粒细胞减少、贫血和血小板减少，伴有大量循环大颗粒淋巴细胞；（3）骨髓穿刺涂片和活检中有大颗粒淋巴细胞浸润；（4）免疫表型：CD2(+)、表面CD3(-)、胞质CD3(+)、CD56(+)、CD57(-)、CD11b(+/-)和CD16(+/-)。无T细胞受体（TCR）基因重排；（5）EB病毒抗体通常呈阳性；（6）无独特的核型异常，有时有6号染色体长臂2区1带至2区5带缺失（del(6)(q21q25)）；（7）排除其他伴有大颗粒淋巴细胞增多的疾病。
