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目的提高对侵袭性NK细胞白血病(ANKL)的认识,对其诊断标准进行总结。方法回顾性分析临床病例。结果和结论ANKL患者常有发热、肝脾肿大、黄疸、肝功能异常和全血细胞减少,疾病进展迅速,短期内出现多脏器功能衰竭、噬血细胞综合征,中位生存期小于2个月。目前较公认的诊断标准:①常有发热及肝、脾、淋巴结肿大;②外周血可以有中性粒细胞减少、贫血和血小板减少,淋巴细胞比例增高,大颗粒淋巴细胞可以增多,但不是诊断的必要条件;③骨髓涂片和活检均可见较多的大颗粒淋巴细胞浸润;④细胞免疫表型为CD2(+),表面CD3(-),胞质CD3(+),CD56(+), CD57(-),CD11b和CD16可以阳性,无T细胞受体重排;⑤有EB病毒抗体阳性的证据;⑥没有特异的染色体异常,较多见的核型异常为de1(6)(q21q25);⑦排除其他引起大颗粒淋巴细胞增多的疾病。
Objective To improve the understanding of aggressive NK cell leukemia (ANKL) and summarize the diagnostic criteria. Methods Retrospective analysis of clinical cases. RESULTS AND CONCLUSIONS: ANKL patients often have fever, hepatosplenomegaly, jaundice, abnormal liver function and pancytopenia. The disease progresses rapidly. In the short term, multiple organ failure and hemophagocytic syndrome occur. The median survival time is less than 2 months . More recognized diagnostic criteria: ① often have fever and liver, spleen, lymph nodes; ② peripheral blood can have neutropenia, anemia and thrombocytopenia, increased proportion of lymphocytes, large particles of lymphocytes can be increased, but not (3) The immunophenotypes of CD2 (+), surface CD3 (-), cytoplasmic CD3 (+), CD56 (+) , CD57 (-), CD11b and CD16 were positive, without T cell receptor rearrangement; ⑤ EBV antibody positive evidence; ⑥ no specific chromosomal abnormalities, more common karyotype abnormalities de1 (6) (q21q25 ) ⑦ exclude other diseases that cause large particles of lymphocytes.