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摘要: 骨髓增殖性肿瘤急变期（blast phase myeloproliferative neoplasm，MPN-BP）是一类继发性急性白血病。MPN-BP患者的预后往往较差，目前尚无标准的治疗方案。其治疗仍是当前临床上的一个重大挑战。近些年来，随着骨髓增殖性肿瘤（myeloproliferative neoplasm，MPN）的生物学机制研究取得重大进展，尤其是驱动基因突变导致JAK-STAT信号通路的异常活化，为MPN-BP的机制研究及治疗手段提供了方向。MPN-BP同原发性急性白血病的发生机制不同，关于MPN-BP的治疗也一直在探索中。本文将重点阐述MPN-BP的治疗进展。Abstract: Blast phase myeloproliferative neoplasm (MPN-BP) represent a type of secondary acute leukemia and are associated with a poor patient prognosis worldwide. Currently, there is no standardized treatment regimen for MPN-BPs, and treatment remains a major clinical challenge. In recent years, significant progress has been made in understanding the biological mechanisms of MPNs, especially the abnormal activation of the JAK-STAT signaling pathway caused by driver gene mutations; this has opened up a new avenue in exploring the mechanism and treatment of MPN-BP. However, in terms of disease pathogenesis, MPN-BP are different from primary acute leukemia, and different therapeutic strategies for MPN-BP have also been investigated. This article focuses on the advances made in MPN-BP treatment.
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Key words:
- myeloproliferative neoplasm (MPN) /
- blast phase /
- treatment /
- leukemia
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表 1 MPN-BP的治疗结果
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