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Blood：Richter综合征的生物学特性和治疗进展

2018-04-26 MedSci MedSci原创

Richter综合征
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Richter综合征（RS）是慢性淋巴细胞白血病（CLL）患者转化或并发另一种侵袭性淋巴瘤。现已识别了RS的两种病理变异型，即弥漫性大B细胞淋巴瘤（DLBCL）变异型和罕见的霍奇金淋巴瘤（HL）变异型。确诊RS需要组织病理检查。临床疑诊RS需根据临床表现和体征。鉴别CLL进展和RS，以及确定活检位点可以依赖PET/CT。总体上，增殖（CDKN2A、NOTCH1、MYC）和凋亡（TP53）调控的分子

Richter综合征（RS）是慢性淋巴细胞白血病（CLL）患者转化或并发另一种侵袭性淋巴瘤。现已识别了RS的两种病理变异型，即弥漫性大B细胞淋巴瘤（DLBCL）变异型和罕见的霍奇金淋巴瘤（HL）变异型。

确诊RS需要组织病理检查。临床疑诊RS需根据临床表现和体征。鉴别CLL进展和RS，以及确定活检位点可以依赖PET/CT。

总体上，增殖（CDKN2A、NOTCH1、MYC）和凋亡（TP53）调控的分子损伤与90%的DLBCL型RS相关，而HL型RS的生物特性基本不明。目前DLBCL型RS的预后不尽如人意，相比HL型RS的预后就稍好一些。

RS最重要的预测因子是CLL和侵袭性淋巴瘤克隆之间的克隆关系，无克隆关联的RS预后更好。

用于治疗DLBCL的包含利妥昔单抗的联合化疗在治疗DLBCL型RS中应用最为广泛。对接受诱导治疗的合适患者予以干细胞移植延长存活期。HL型RS首选ABVD方案，而且在这种情况下，较少再进行干细胞移植巩固。

原始出处：

Davide Rossi， Valeria Spina， and Gianluca Gaidano.Biology and treatment of Richter syndrome.Blood  2018  ：blood-2018-01-791376；  doi： https：//doi.org/10.1182/blood-2018-01-791376

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    2018-05-21 liuquanmn

    #Richter综合征#

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    2018-04-28 diushouji

    #ICH#

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    2018-04-28 zhouqu_8

    #综合征#

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    2018-04-28 12498c32m74暂无昵称

    #生物学#

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  5. [GetPortalCommentsPageByObjectIdResponse(id=1927584, encodeId=5e62192e5844f, content=<a href='/topic/show?id=f6ff154653c' target=_blank style='color:#2F92EE;'>#Richter综合征#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=33, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=15465, encryptionId=f6ff154653c, topicName=Richter综合征)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=9c94141, createdName=liuquanmn, createdTime=Mon May 21 06:23:00 CST 2018, time=2018-05-21, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1316354, encodeId=92a3131635462, content=<a href='/topic/show?id=f3789349c3' target=_blank style='color:#2F92EE;'>#ICH#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=27, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=9349, encryptionId=f3789349c3, topicName=ICH)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c192448, createdName=diushouji, createdTime=Sat Apr 28 06:23:00 CST 2018, time=2018-04-28, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1431199, encodeId=81221431199cd, content=<a href='/topic/show?id=f9b9e9124a8' target=_blank style='color:#2F92EE;'>#综合征#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=18, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=79124, encryptionId=f9b9e9124a8, topicName=综合征)], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/aLGWoFXAyMbIu3qymFOyheQLjPSX3OUs5GmkyBlcCOwTPIeq3why9NGibxxUqYo6hcx8qZLHZFgNPnBK1yzWeOFpyg2OnWOt0/0, createdBy=fa4716, createdName=zhouqu_8, createdTime=Sat Apr 28 06:23:00 CST 2018, time=2018-04-28, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1617862, encodeId=ad23161e86231, content=<a href='/topic/show?id=e88f6930185' target=_blank style='color:#2F92EE;'>#生物学#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=32, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=69301, encryptionId=e88f6930185, topicName=生物学)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=e44019811593, createdName=12498c32m74暂无昵称, createdTime=Sat Apr 28 06:23:00 CST 2018, time=2018-04-28, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=309519, encodeId=92cd30951963, content=学习.谢谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=66, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/aLGWoFXAyMbIu3qymFOyhb9xDeHojCicsdRdyC3XXdGk2ib9yLypZuHN06WeW4HO8WN0GGrgEfvnTX8gsckof05GHOU5OM4Mvx/0, createdBy=9cee1982656, createdName=执着追梦, createdTime=Thu Apr 26 17:34:07 CST 2018, time=2018-04-26, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=309503, encodeId=d5713095037a, content=谢谢分享学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=68, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/Q3auHgzwzM7GibvIBibibnnnvpajk2stlDF5oichYahI6Bia8uxeTBFoPbdoHWebyXf9eal9FNgUF2lNcrwoZicM3BvQ/0, createdBy=a3742066385, createdName=戒馋，懒，贪, createdTime=Thu Apr 26 15:52:56 CST 2018, time=2018-04-26, status=1, ipAttribution=)]
    2018-04-26 执着追梦

    学习.谢谢分享

    0

  6. [GetPortalCommentsPageByObjectIdResponse(id=1927584, encodeId=5e62192e5844f, content=<a href='/topic/show?id=f6ff154653c' target=_blank style='color:#2F92EE;'>#Richter综合征#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=33, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=15465, encryptionId=f6ff154653c, topicName=Richter综合征)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=9c94141, createdName=liuquanmn, createdTime=Mon May 21 06:23:00 CST 2018, time=2018-05-21, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1316354, encodeId=92a3131635462, content=<a href='/topic/show?id=f3789349c3' target=_blank style='color:#2F92EE;'>#ICH#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=27, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=9349, encryptionId=f3789349c3, topicName=ICH)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c192448, createdName=diushouji, createdTime=Sat Apr 28 06:23:00 CST 2018, time=2018-04-28, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1431199, encodeId=81221431199cd, content=<a href='/topic/show?id=f9b9e9124a8' target=_blank style='color:#2F92EE;'>#综合征#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=18, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=79124, encryptionId=f9b9e9124a8, topicName=综合征)], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/aLGWoFXAyMbIu3qymFOyheQLjPSX3OUs5GmkyBlcCOwTPIeq3why9NGibxxUqYo6hcx8qZLHZFgNPnBK1yzWeOFpyg2OnWOt0/0, createdBy=fa4716, createdName=zhouqu_8, createdTime=Sat Apr 28 06:23:00 CST 2018, time=2018-04-28, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1617862, encodeId=ad23161e86231, content=<a href='/topic/show?id=e88f6930185' target=_blank style='color:#2F92EE;'>#生物学#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=32, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=69301, encryptionId=e88f6930185, topicName=生物学)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=e44019811593, createdName=12498c32m74暂无昵称, createdTime=Sat Apr 28 06:23:00 CST 2018, time=2018-04-28, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=309519, encodeId=92cd30951963, content=学习.谢谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=66, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/aLGWoFXAyMbIu3qymFOyhb9xDeHojCicsdRdyC3XXdGk2ib9yLypZuHN06WeW4HO8WN0GGrgEfvnTX8gsckof05GHOU5OM4Mvx/0, createdBy=9cee1982656, createdName=执着追梦, createdTime=Thu Apr 26 17:34:07 CST 2018, time=2018-04-26, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=309503, encodeId=d5713095037a, content=谢谢分享学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=68, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/Q3auHgzwzM7GibvIBibibnnnvpajk2stlDF5oichYahI6Bia8uxeTBFoPbdoHWebyXf9eal9FNgUF2lNcrwoZicM3BvQ/0, createdBy=a3742066385, createdName=戒馋，懒，贪, createdTime=Thu Apr 26 15:52:56 CST 2018, time=2018-04-26, status=1, ipAttribution=)]
    2018-04-26 戒馋，懒，贪

    谢谢分享学习了

    0