毛细胞白血病的诊断与治疗新进展

International Journal of Hematologic Oncology Pub Date : 2015-11-12 DOI:10.2217/IJH.15.23

Asad Javed, Upasana Joneja, J. Gong, G. Uppal

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引用次数: 0

摘要

毛细胞白血病(HCL)是一种罕见的惰性b细胞增生性疾病，占所有白血病病例的2%。大多数患者表现为全血细胞减少和脾肿大，血液中有不同数量的“毛状”淋巴细胞。BRAF V600E突变几乎可以在100%的HCL病例中检测到，而在其他b细胞淋巴瘤中则不存在。突变基因及其对应的异常蛋白可作为诊断HCL的特异性标志物。针对突变BRAF及其下游通路的新治疗方式在临床试验中显示出令人鼓舞的结果。本文就毛细胞白血病的诊断和治疗的最新进展作一综述。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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Recent advances in diagnosis and treatment of hairy cell leukemia

Hairy cell leukemia (HCL) is a rare, indolent B-cell lymphoproliferative disorder that accounts for 2% of all cases of leukemia. Most patients present with pancytopenia and splenomegaly with variable number of ‘hairy’ lymphocytes in blood. BRAF V600E mutation can be detected in virtually 100% of HCL cases and is absent in other B-cell lymphomas. The mutated gene and its responding abnormal protein can be used as specific markers in the diagnosis of HCL. New therapeutic modalities targeting on mutated BRAF and its downstream pathways have shown encouraging results in clinical trials. The objective of this review article is to discuss the recent developments in the diagnosis and management of hairy cell leukemia.

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来源期刊

International Journal of Hematologic Oncology

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0.00%

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审稿时长

13 weeks

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