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目的提高对侵袭性自然杀伤细胞白血病(ANKL)的认识,探讨早期诊断及有效的治疗方法。方法回顾性分析四川大学华西医院血液科2006年3月至2009年10月收治的14例ANKL患者临床资料并进行文献复习。结果 14例患者中男11例,女3例,中位年龄23岁(15～47岁)。患者初诊时发热14例(100%),浅表淋巴结肿大9例(64.3%),肝脏肿大13例(92.9%),脾脏肿大14例(100%)。全血细胞减少7例(50%),骨髓浸润14例(100%),肝功能受损13例(92.9%),低纤维蛋白原血症9例(64.3%)。细胞免疫表型为CD2(+)100%,CD16(+)64.3%,CD56(+)92.9%,sCD3(-)100%,cCD3(+)55.6%,CD7(+)61.5%,CD57(+)9.1%。9例人类疱疹病毒(EBV)检查的患者中7例阳性(77.8%)。患者中位生存期10周。结论 ANKL是一种少见类型的白血病,对化疗不敏感,进展迅速,预后极差。几乎所有患者均有骨髓浸润,骨髓细胞形态学和流式细胞学检查对本病的诊断具有重要价值。
Objective To improve the understanding of invasive natural killer cell leukemia (ANKL) and explore early diagnosis and effective treatment. Methods The clinical data of 14 patients with ANKL admitted to Department of Hematology, Huaxi Hospital, Sichuan University from March 2006 to October 2009 were reviewed retrospectively. Results Of the 14 patients, 11 were male and 3 were female, with a median age of 23 years (15-47 years). In the first visit, 14 cases (100%) had fever, 9 cases (64.3%) had superficial lymph nodes, 13 cases (92.9%) had liver enlargement and 14 cases (100%) had enlarged spleen. Complete pancytopenia was found in 7 (50%), bone marrow infiltration in 14 (100%), impaired liver function in 13 (92.9%) and hypofibrinogenemia in 9 (64.3%). Cell immunophenotypes were 100% for CD2 (+), 64.3% for CD16 (+), 92.9% for CD56 (+), 100% for sCD3 (-), 55.6% for cCD3 (+), 61.5% for CD7 (+), ) 9.1%. Seven of the nine patients with human herpesvirus (EBV) were positive (77.8%). The median survival of patients was 10 weeks. Conclusion ANKL is a rare type of leukemia, which is not sensitive to chemotherapy and has a rapid progression with poor prognosis. Almost all patients have bone marrow infiltration, bone marrow cell morphology and flow cytometry of the diagnosis of the disease is of great value.