Department of Pediatric Hematology and Oncology, the Affiliated Hospital of Qingdao University, Qingdao, Shandong, China.
Department of Respiratory & Cardiovascular Pediatrics, the Affiliated Hospital of Qingdao University, Qingdao, Shandong, China.
Turk J Pediatr. 2024 Oct 7;66(4):481-489. doi: 10.24953/turkjpediatr.2024.5072.
Aggressive natural killer cell leukemia (ANKL) is rare and difficult to diagnose in early stages, with no standard treatment and a poor prognosis.
Two adolescents with ANKL presented with hemophagocytic lymphohistiocytosis (HLH), with Case-1 presenting as refractory HLH and Case-2 with lung involvement. The morphology of bone marrow showed an increase in unidentified cells, which mainly expressed CD56. Cytogenetic analysis showed complex karyotypes. Both patients received intensive combined chemotherapy based on pegaspargase and anthracyclines. Case-1 died of tumor lysis syndrome. Case-2 underwent hematopoietic stem cell transplantation and is currently alive and disease-free.
HLH can serve as the initial manifestation of ANKL. Leukemia cells of ANKL have significant variations in the morphology and mainly express CD56. Intensive combination chemotherapy based on pegaspargase and anthracyclines may be considered for ANKL.
侵袭性自然杀伤细胞白血病（ANKL）较为罕见，早期诊断困难，缺乏标准治疗方案，预后较差。
两名青少年患者均以噬血细胞性淋巴组织细胞增生症（HLH）起病，其中病例 1 为难治性 HLH，病例 2 合并肺部受累。骨髓形态学表现为不明原因细胞增多，主要表达 CD56。细胞遗传学分析显示复杂核型。两名患者均接受基于培门冬酶和蒽环类药物的强化联合化疗。病例 1 死于肿瘤溶解综合征。病例 2 行造血干细胞移植后，目前无病生存。
HLH 可作为 ANKL 的首发表现。ANKL 的白血病细胞形态学变化较大，主要表达 CD56。基于培门冬酶和蒽环类药物的强化联合化疗可用于治疗 ANKL。
