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Blood：用JAK1/2抑制剂治疗骨髓增生性肿瘤（MPN）可增加患者恶性B细胞淋巴瘤的发病率

2018-06-18 MedSci MedSci原创

JAK1/2抑制剂骨髓增生性肿瘤B细胞淋巴瘤
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抑制Janus激酶1/2(JAK1/2）是治疗骨髓增生性肿瘤（MPN）的主要方法。散发观察报道JAK1/2抑制剂治疗MPN过程中可共发B细胞非霍奇金淋巴瘤。Edit Porpaczy等人对626例MPN患者进行评估，其中包括69位采用JAK1/2抑制剂治疗的骨髓纤维化患者。69位采用JAK1/2治疗的患者中有4位（5.8%）进展成B细胞淋巴瘤，而采用常规治疗的557位患者中仅有2位（0.36%），

抑制Janus激酶1/2(JAK1/2）是治疗骨髓增生性肿瘤（MPN）的主要方法。散发观察报道JAK1/2抑制剂治疗MPN过程中可共发B细胞非霍奇金淋巴瘤。Edit Porpaczy等人对626例MPN患者进行评估，其中包括69位采用JAK1/2抑制剂治疗的骨髓纤维化患者。

69位采用JAK1/2治疗的患者中有4位（5.8%）进展成B细胞淋巴瘤，而采用常规治疗的557位患者中仅有2位（0.36%），风险增加16倍。在另外一个独立的MPN患者（929位）队列中风险增加15倍。仅考虑原发性骨髓瘤患者（216位），31例抑制剂治疗的患者中有3位进展成淋巴瘤（9.7%），而对照的185位中仅1位（0.54%）。发生JAK2 V617F或其他MPN相关突变时，淋巴瘤呈侵袭性B细胞型、淋巴结外或白血病，伴有高MYC表达。

从开始抑制剂治疗到确诊淋巴瘤的中位时间是25个月。在16.3%的患者骨髓纤维化时的骨髓中检测到克隆性免疫球蛋白基因重组。在JAK1/2抑制剂治疗期间发展的淋巴瘤受已存在的B细胞克隆导向，在3位检测的患者中均是如此。

对2位患者进行测序验证了克隆基因的存在。Stat-/-小鼠可模拟JAK1/2抑制剂效应：16/24小鼠出现自发性髓系增生，并伴有异常B细胞。患病小鼠骨髓移植揭示恶性B细胞克隆生长进展成侵袭性B细胞白血病-淋巴瘤。

总而言之，本研究结果提示抑制骨髓纤维化个体的JAK/STAT1信号通路可增加侵袭性B细胞淋巴瘤的发病率。检测是否存在B细胞克隆或可鉴定高风险个体。

原始出处：

Edit Porpaczy，et al. Aggressive B-cell lymphomas in patients with myelofibrosis receiving JAK1/2 inhibitor therapy. Blood  2018  ：blood-2017-10-810739；  doi： https：//doi.org/10.1182/blood-2017-10-810739

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    2019-04-26 12498568m50暂无昵称

    #MPN#

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    2018-11-19 jklm09

    #抑制剂#

    0

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createdTime=Wed Jan 30 20:11:00 CST 2019, time=2019-01-30, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1995530, encodeId=4b681995530d9, content=<a href='/topic/show?id=992411149e' target=_blank style='color:#2F92EE;'>#2抑制剂#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=80, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=1114, encryptionId=992411149e, topicName=2抑制剂)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=28b7142, createdName=liuquan, createdTime=Fri Jan 18 06:11:00 CST 2019, time=2019-01-18, status=1, ipAttribution=)]
    2019-01-17 fengyqf

    #骨髓增生#

    0

  4. 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encodeId=96293490939a, content=学习了，谢谢作者分享!, beContent=null, objectType=article, channel=null, level=null, likeNumber=108, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mONcle9pic3xJXLtMtt5B40XXuPjn9xn1rBhrM6fHLkX1hI86ZoWoPJkgDkZuJNKkBfBHOcRd1fAdezg7JlHNOokohdAp3WkI/0, createdBy=32c81964262, createdName=1e145228m78(暂无匿称), createdTime=Sat Oct 13 05:22:32 CST 2018, time=2018-10-13, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1757310, encodeId=a5221e57310ea, content=<a href='/topic/show?id=2f354231613' target=_blank style='color:#2F92EE;'>#增生性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=76, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=42316, encryptionId=2f354231613, topicName=增生性)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=1f7c37300907, createdName=ms7514935887898259, createdTime=Wed Jan 30 20:11:00 CST 2019, time=2019-01-30, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1995530, encodeId=4b681995530d9, content=<a href='/topic/show?id=992411149e' target=_blank style='color:#2F92EE;'>#2抑制剂#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=80, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=1114, encryptionId=992411149e, topicName=2抑制剂)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=28b7142, createdName=liuquan, createdTime=Fri Jan 18 06:11:00 CST 2019, time=2019-01-18, status=1, ipAttribution=)]
    2018-09-06 lifestar

    #发病率#

    0

  5. 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createdTime=Wed Jan 30 20:11:00 CST 2019, time=2019-01-30, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1995530, encodeId=4b681995530d9, content=<a href='/topic/show?id=992411149e' target=_blank style='color:#2F92EE;'>#2抑制剂#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=80, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=1114, encryptionId=992411149e, topicName=2抑制剂)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=28b7142, createdName=liuquan, createdTime=Fri Jan 18 06:11:00 CST 2019, time=2019-01-18, status=1, ipAttribution=)]
    2018-10-11 marlenexl

    #细胞淋巴瘤#

    0

  6. 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createdTime=Wed Jan 30 20:11:00 CST 2019, time=2019-01-30, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1995530, encodeId=4b681995530d9, content=<a href='/topic/show?id=992411149e' target=_blank style='color:#2F92EE;'>#2抑制剂#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=80, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=1114, encryptionId=992411149e, topicName=2抑制剂)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=28b7142, createdName=liuquan, createdTime=Fri Jan 18 06:11:00 CST 2019, time=2019-01-18, status=1, ipAttribution=)]
    2018-10-25 12293217m86暂无昵称

    看来治疗之后还要注意淋巴瘤发生

    0

  7. 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createdTime=Wed Jan 30 20:11:00 CST 2019, time=2019-01-30, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1995530, encodeId=4b681995530d9, content=<a href='/topic/show?id=992411149e' target=_blank style='color:#2F92EE;'>#2抑制剂#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=80, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=1114, encryptionId=992411149e, topicName=2抑制剂)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=28b7142, createdName=liuquan, createdTime=Fri Jan 18 06:11:00 CST 2019, time=2019-01-18, status=1, ipAttribution=)]
    2018-08-19 1249859em38(暂无昵称)

    #JAK1/2抑制剂#

    0

  8. 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createdTime=Wed Jan 30 20:11:00 CST 2019, time=2019-01-30, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1995530, encodeId=4b681995530d9, content=<a href='/topic/show?id=992411149e' target=_blank style='color:#2F92EE;'>#2抑制剂#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=80, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=1114, encryptionId=992411149e, topicName=2抑制剂)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=28b7142, createdName=liuquan, createdTime=Fri Jan 18 06:11:00 CST 2019, time=2019-01-18, status=1, ipAttribution=)]
    2018-10-13 1e145228m78(暂无匿称)

    学习了，谢谢作者分享!

    0

  9. 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createdTime=Wed Jan 30 20:11:00 CST 2019, time=2019-01-30, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1995530, encodeId=4b681995530d9, content=<a href='/topic/show?id=992411149e' target=_blank style='color:#2F92EE;'>#2抑制剂#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=80, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=1114, encryptionId=992411149e, topicName=2抑制剂)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=28b7142, createdName=liuquan, createdTime=Fri Jan 18 06:11:00 CST 2019, time=2019-01-18, status=1, ipAttribution=)]
    2019-01-30 ms7514935887898259

    #增生性#

    0

  10. 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    2019-01-18 liuquan

    #2抑制剂#

    0

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