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IJLH：应重新评估将难治性贫血作为MDS/MPN‐RS‐T诊断标准的要求？

2019-07-22 Gladiator MedSci原创

贫血骨髓增生异常
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骨髓增生异常/骨髓增生性肿瘤会伴有环状核母细胞增生和血小板增多(MDS/MPN‐RS‐T)，这种疾病是2016年世界卫生组织(WHO)修订分类中的一种罕见疾病。诊断标准包括：持续性血小板增多症(>450×109/L)伴不典型巨核细胞聚集，难治疗性贫血，伴有环状核母细胞的浆细胞发育不良，存在剪接体因子3b亚单位(SF3B1)突变。目前还不清楚贫血是否应该作为诊断的必要标准，因为某些病例显示了M

骨髓增生异常/骨髓增生性肿瘤会伴有环状核母细胞增生和血小板增多(MDS/MPN‐RS‐T)，这种疾病是2016年世界卫生组织(WHO)修订分类中的一种罕见疾病。诊断标准包括：持续性血小板增多症(>450×109/L)伴不典型巨核细胞聚集，难治疗性贫血，伴有环状核母细胞的浆细胞发育不良，存在剪接体因子3b亚单位(SF3B1)突变。目前还不清楚贫血是否应该作为诊断的必要标准，因为某些病例显示了MDS/MPN‐RS‐T的所有其他特征，但没有出现贫血。

研究人员在两个主要的学术机构中寻找了诊断为难治性贫血的MDS/MPN‐RS‐T的临界病例。确诊3例无贫血病例。这些病例均表现出MDS/MPN‐RS‐T的其他经典形态学和临床特征，包括血小板增多症、聚类的非典型巨核细胞以及SF3B1和JAK2 V617F的特征性突变。

鉴于这些发现，应重新评估将难治性贫血作为MDS/MPN‐RS‐T诊断标准的要求。排除难治性贫血作为诊断标准将纳入当前的边缘性病例并扩大该疾病的范围。

原始出处：

Peng Li, Nahid Shahmarvand ,Revisiting diagnostic criteria for myelodysplastic/myeloproliferative neoplasms with ring sideroblasts and thrombocytosis: Borderline cases without anemia exist

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  1. [GetPortalCommentsPageByObjectIdResponse(id=1842807, encodeId=b791184280eea, content=<a href='/topic/show?id=9f061142249' target=_blank style='color:#2F92EE;'>#MDS#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=54, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=11422, encryptionId=9f061142249, topicName=MDS)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=80a244, createdName=d830372, createdTime=Mon Feb 03 22:55:00 CST 2020, time=2020-02-03, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1935495, encodeId=9e1a193549592, content=<a href='/topic/show?id=4302121e605' target=_blank style='color:#2F92EE;'>#MPN#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=48, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=12176, encryptionId=4302121e605, topicName=MPN)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=1e452500029, createdName=12498568m50暂无昵称, createdTime=Sun Oct 06 06:55:00 CST 2019, time=2019-10-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1385030, encodeId=7ad713850307d, content=<a href='/topic/show?id=354691625f1' target=_blank style='color:#2F92EE;'>#诊断标准#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=45, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=91625, encryptionId=354691625f1, topicName=诊断标准)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=3516494, createdName=spoonycyy, createdTime=Wed Jul 24 00:55:00 CST 2019, time=2019-07-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1528592, encodeId=4b191528592c8, content=<a href='/topic/show?id=74b798601a1' target=_blank style='color:#2F92EE;'>#难治性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=42, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=98601, encryptionId=74b798601a1, topicName=难治性)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=a1ef11916292, createdName=freve, createdTime=Wed Jul 24 00:55:00 CST 2019, time=2019-07-24, status=1, ipAttribution=)]
    2020-02-03 d830372

    #MDS#

    0

  2. [GetPortalCommentsPageByObjectIdResponse(id=1842807, encodeId=b791184280eea, content=<a href='/topic/show?id=9f061142249' target=_blank style='color:#2F92EE;'>#MDS#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=54, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=11422, encryptionId=9f061142249, topicName=MDS)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=80a244, createdName=d830372, createdTime=Mon Feb 03 22:55:00 CST 2020, time=2020-02-03, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1935495, encodeId=9e1a193549592, content=<a href='/topic/show?id=4302121e605' target=_blank style='color:#2F92EE;'>#MPN#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=48, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=12176, encryptionId=4302121e605, topicName=MPN)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=1e452500029, createdName=12498568m50暂无昵称, createdTime=Sun Oct 06 06:55:00 CST 2019, time=2019-10-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1385030, encodeId=7ad713850307d, content=<a href='/topic/show?id=354691625f1' target=_blank style='color:#2F92EE;'>#诊断标准#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=45, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=91625, encryptionId=354691625f1, topicName=诊断标准)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=3516494, createdName=spoonycyy, createdTime=Wed Jul 24 00:55:00 CST 2019, time=2019-07-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1528592, encodeId=4b191528592c8, content=<a href='/topic/show?id=74b798601a1' target=_blank style='color:#2F92EE;'>#难治性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=42, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=98601, encryptionId=74b798601a1, topicName=难治性)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=a1ef11916292, createdName=freve, createdTime=Wed Jul 24 00:55:00 CST 2019, time=2019-07-24, status=1, ipAttribution=)]
    2019-10-06 12498568m50暂无昵称

    #MPN#

    0

  3. [GetPortalCommentsPageByObjectIdResponse(id=1842807, encodeId=b791184280eea, content=<a href='/topic/show?id=9f061142249' target=_blank style='color:#2F92EE;'>#MDS#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=54, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=11422, encryptionId=9f061142249, topicName=MDS)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=80a244, createdName=d830372, createdTime=Mon Feb 03 22:55:00 CST 2020, time=2020-02-03, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1935495, encodeId=9e1a193549592, content=<a href='/topic/show?id=4302121e605' target=_blank style='color:#2F92EE;'>#MPN#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=48, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=12176, encryptionId=4302121e605, topicName=MPN)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=1e452500029, createdName=12498568m50暂无昵称, createdTime=Sun Oct 06 06:55:00 CST 2019, time=2019-10-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1385030, encodeId=7ad713850307d, content=<a href='/topic/show?id=354691625f1' target=_blank style='color:#2F92EE;'>#诊断标准#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=45, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=91625, encryptionId=354691625f1, topicName=诊断标准)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=3516494, createdName=spoonycyy, createdTime=Wed Jul 24 00:55:00 CST 2019, time=2019-07-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1528592, encodeId=4b191528592c8, content=<a href='/topic/show?id=74b798601a1' target=_blank style='color:#2F92EE;'>#难治性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=42, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=98601, encryptionId=74b798601a1, topicName=难治性)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=a1ef11916292, createdName=freve, createdTime=Wed Jul 24 00:55:00 CST 2019, time=2019-07-24, status=1, ipAttribution=)]
    2019-07-24 spoonycyy

    #诊断标准#

    0

  4. [GetPortalCommentsPageByObjectIdResponse(id=1842807, encodeId=b791184280eea, content=<a href='/topic/show?id=9f061142249' target=_blank style='color:#2F92EE;'>#MDS#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=54, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=11422, encryptionId=9f061142249, topicName=MDS)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=80a244, createdName=d830372, createdTime=Mon Feb 03 22:55:00 CST 2020, time=2020-02-03, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1935495, encodeId=9e1a193549592, content=<a href='/topic/show?id=4302121e605' target=_blank style='color:#2F92EE;'>#MPN#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=48, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=12176, encryptionId=4302121e605, topicName=MPN)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=1e452500029, createdName=12498568m50暂无昵称, createdTime=Sun Oct 06 06:55:00 CST 2019, time=2019-10-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1385030, encodeId=7ad713850307d, content=<a href='/topic/show?id=354691625f1' target=_blank style='color:#2F92EE;'>#诊断标准#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=45, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=91625, encryptionId=354691625f1, topicName=诊断标准)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=3516494, createdName=spoonycyy, createdTime=Wed Jul 24 00:55:00 CST 2019, time=2019-07-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1528592, encodeId=4b191528592c8, content=<a href='/topic/show?id=74b798601a1' target=_blank style='color:#2F92EE;'>#难治性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=42, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=98601, encryptionId=74b798601a1, topicName=难治性)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=a1ef11916292, createdName=freve, createdTime=Wed Jul 24 00:55:00 CST 2019, time=2019-07-24, status=1, ipAttribution=)]
    2019-07-24 freve

    #难治性#

    0

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