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致命性高白细胞性T淋巴母细胞淋巴瘤/白血病合并多基因融合与突变:临床启示与感悟

Fatal hyperleukocytic T lymphoblastic lymphoma/leukemia complicated with multi-gene fusion and mutation: clinical revelation and perception.

作者信息

Liu Yanquan, Issah Mohammed Awal, Hu Xiaomei, Shen Jianzhen

机构信息

Fujian Institute of Hematology, National and Fujian Provincial Key Laboratory of Hematology, Department of Hematology of Fujian Medical University Union Hospital NO.29 Xinquan Road, Gulou District, Fuzhou 350001, Fujian Province, China.

Department of Pathology of Fujian Medical University Union Hospital NO.29 Xinquan Road, Gulou District, Fuzhou 350001, Fujian Province, China.

出版信息

Am J Blood Res. 2020 Dec 15;10(6):440-446. eCollection 2020.

Abstract

T lymphoblastic lymphoma/leukemia (T-LBL/ALL) is a highly malignant hematological tumor common in young males. Most T-LBL/ALL patients usually initially seek medical treatment for clinical manifestations of non-hematological diseases. Presently, T-ALL chemotherapy is often used for the treatment of T-LBL/ALL internationally. With the application of high-intensity standard chemotherapy, the efficacy and prognosis of T-LBL/ALL are still not optimistic. The authors present a young male patient with facial and neck edema as the initial symptoms. This young patient of T-LBL/ALL was found to have a mediastinal mass after CT examination and he was finally diagnosed as highly malignant T-LBL/ALL. Unfortunately, after undergoing three standard courses of high-intensity chemotherapy, the young male patient eventually died of white blood cell stasis and severe infection caused by hyperleukocytosis. To this end, we find that the prognosis of T-LBL/ALL with multiple gene mutations or fusions and hyperleukocytosis, is extremely poor, and probably becomes a medical problem worthy of continuing resolution in the field of hematology and oncology.

摘要

T淋巴母细胞淋巴瘤/白血病(T-LBL/ALL)是一种常见于年轻男性的高度恶性血液肿瘤。大多数T-LBL/ALL患者最初通常因非血液系统疾病的临床表现而就医。目前,国际上T-ALL化疗常被用于治疗T-LBL/ALL。随着高强度标准化化疗的应用,T-LBL/ALL的疗效和预后仍然不容乐观。作者介绍了一名以面部和颈部水肿为首发症状的年轻男性患者。这位T-LBL/ALL的年轻患者经CT检查发现纵隔肿块,最终被诊断为高度恶性T-LBL/ALL。不幸的是,在接受了三个疗程的高强度标准化化疗后,这位年轻男性患者最终死于白细胞淤滞和高白细胞血症引起的严重感染。为此,我们发现伴有多种基因突变或融合以及高白细胞血症的T-LBL/ALL预后极差,可能成为血液学和肿瘤学领域值得持续解决的医学问题。

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本文引用的文献

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Loss of KDM6A confers drug resistance in acute myeloid leukemia.KDM6A 的缺失赋予急性髓系白血病的耐药性。
Leukemia. 2020 Jan;34(1):50-62. doi: 10.1038/s41375-019-0497-6. Epub 2019 Jun 14.
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Hyperleukocytosis and leukostasis: management of a medical emergency.高白细胞血症和白细胞淤滞症:医疗急症处理。
Expert Rev Hematol. 2017 Feb;10(2):147-154. doi: 10.1080/17474086.2017.1270754. Epub 2016 Dec 26.

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