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学科主题临床医学
Adult Aggressive Natural Killer Cell Leukemia
Zhang, Hongyu1; Meng, Qinxiang1; Yin, Weihua2; Xu, Lei1; Lie, Liping3
关键词Aggressive natural killer cell leukemia Prognosis Bone marrow biopsy Immunohistochemistry
刊名AMERICAN JOURNAL OF THE MEDICAL SCIENCES
2013-07-01
346期:1页:56-63
收录类别SCI
文章类型Article
WOS标题词Science & Technology
类目[WOS]Medicine, General & Internal
研究领域[WOS]General & Internal Medicine
关键词[WOS]GRANULAR LYMPHOCYTE LEUKEMIA ; OF-THE-LITERATURE ; MALIGNANCIES ; GEMCITABINE ; LYMPHOMAS
英文摘要

Introduction: Aggressive natural killer cell leukemia (ANKL) is a rare malignancy with a poor prognosis. Methods: Clinical and laboratory data of 20 patients with ANKL were collected retrospectively from a single medical center. Results: The prominent clinical complications included unexplained fever, interstitial pneumonia, hepatosplenomegaly, high level of lactate dehydrogenase and liver dysfunction. Both bone marrow (BM) biopsies and aspiration of 20 patients were morphologically and immunophenotypically evaluated. Only 2 patients with marked elevation of peripheral large granular lymphocytes had sufficient BM aspiration for flow cytometric immunophenotyping. However, 20 BM biopsies showed marked neoplastic cell infiltration, and immunohistochemistry highlighted patterns of neoplastic involvement. The neoplastic cells were mainly positive for CD2, CD56, CD3(C)(+) and Epstein-Barr virus (EBV)-encoded RNA. The plasma EBV-DNA test was positive in all cases, and complex cytogenetic abnormalities were identified in 8 cases. The median survival of the patients was 8 weeks, and the presence of liver dysfunction-induced jaundice (serum total bilirubin level > 34.2 mu mol/L) was identified as a risk factor for early death. Patients were generally resistant to chemotherapy, but notably, 3 patients demonstrated a marked response to gemcitabine with tolerable toxicities. Conclusions: These findings indicate that ANKL is a highly aggressive leukemia with a fulminating clinical course. A BM biopsy with immunohistochemistry and EBV-encoded RNA detection is mandatory for a rapid diagnosis of ANKL. Therefore, a careful evaluation and fully supported treatment plan should be conducted before chemotherapy is administered to patients with severe complications to improve the overall survival.

语种英语
WOS记录号WOS:000330432900009
引用统计
被引频次:6[WOS]   [WOS记录]     [WOS相关记录]
文献类型期刊论文
条目标识符http://ir.bjmu.edu.cn/handle/400002259/59772
专题北京大学深圳医院_血液内科
北京大学第三临床医学院_放射科
北京大学第三临床医学院_呼吸科
作者单位1.Peking Univ, ShenZhen Hosp, Dept Hematol, Shenzhen 518036, Peoples R China
2.Peking Univ, ShenZhen Hosp, Dept Pathol, Shenzhen 518036, Peoples R China
3.Peking Univ, ShenZhen Hosp, Dept Immunol, Shenzhen 518036, Peoples R China
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Zhang, Hongyu,Meng, Qinxiang,Yin, Weihua,et al. Adult Aggressive Natural Killer Cell Leukemia[J]. AMERICAN JOURNAL OF THE MEDICAL SCIENCES,2013,346(1):56-63.
APA Zhang, Hongyu,Meng, Qinxiang,Yin, Weihua,Xu, Lei,&Lie, Liping.(2013).Adult Aggressive Natural Killer Cell Leukemia.AMERICAN JOURNAL OF THE MEDICAL SCIENCES,346(1),56-63.
MLA Zhang, Hongyu,et al."Adult Aggressive Natural Killer Cell Leukemia".AMERICAN JOURNAL OF THE MEDICAL SCIENCES 346.1(2013):56-63.
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