侵袭性自然杀伤细胞白血病的临床病理特征和治疗:病例系列和文献综述。

Yongan Ni, Lei Li, Yuping Wang, Lirong Sun
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引用次数: 0

摘要

背景:侵袭性自然杀伤细胞白血病(ANKL侵袭性自然杀伤细胞白血病(ANKL)非常罕见,早期诊断困难,没有标准治疗方法,预后较差:两名患有ANKL的青少年出现了嗜血细胞淋巴组织细胞增多症(HLH),病例1表现为难治性HLH,病例2累及肺部。骨髓形态显示不明细胞增多,主要表达 CD56。细胞遗传学分析显示核型复杂。两名患者都接受了基于培加司琼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接受了造血干细胞移植,目前存活无病:结论:HLH可作为ANKL的初始表现。结论:HLH可作为ANKL的初始表现,ANKL的白血病细胞在形态上有显著差异,主要表达CD56。对于ANKL,可以考虑基于培加司琼酶和蒽环类药物的强化联合化疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Clinicopathological features and treatment of aggressive natural killer cell leukemia: case series and literature review.

Background: Aggressive natural killer cell leukemia (ANKL) is rare and difficult to diagnose in early stages, with no standard treatment and a poor prognosis.

Case presentation: 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.

Conclusions: 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.

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