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侵袭性 NK 细胞白血病的临床病理特征与治疗:病例系列与文献综述

英文原题:Clinicopathological features and treatment of aggressive natural killer cell leukemia: case series and literature review.

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Clinicopathological features and treatment of aggressive natural killer cell leukemia: case series and literature review.

PubMed 2024/10/07(内容时间) Turk J Pediatr Q3 · IF 0.9(JCR 2025)

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研究概要

HLH 可作为 ANKL 的首发表现。

中文摘要

侵袭性 NK 细胞白血病(ANKL)罕见,早期诊断困难,目前没有标准治疗,预后不良。病例介绍:两名 ANKL 青少年患者均以噬血细胞性淋巴组织细胞增多症(HLH)就诊;病例 1 表现为难治性 HLH,病例 2 有肺部受累。骨髓形态学显示未明确分类细胞增多,主要表达 CD56。细胞遗传学分析显示复杂核型。两名患者均接受以 pegaspargase 和蒽环类药物为基础的强化联合化疗。病例 1 死于肿瘤溶解综合征。病例 2 接受造血干细胞移植,目前存活且无病。

HLH 可作为 ANKL 的初始表现。ANKL 白血病细胞形态存在明显差异,且主要表达 CD56。可考虑对 ANKL 采用以 pegaspargase 和蒽环类药物为基础的强化联合化疗。

展开英文摘要原文

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.

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.

论文信息

作者
Ni Y、Li L、Wang Y、Sun L
单位
Department of Pediatric Hematology and Oncology, the Affiliated Hospital of Qingdao University, Qingdao, Shandong, China.China
文献类型
病例报告 · 综述
期刊
The Turkish journal of pediatrics2024 Oct 7
原文标识
PubMed 39387420 · DOI 10.24953/turkjpediatr.2024.5072