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NEJM：巨球蛋白血症致视网膜病变-案例报道

2016-01-07 chenshuo译 MedSci原创

巨球蛋白视网膜眼底镜检查
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http://www.nejm.org/doi/full/10.1056/NEJMicm1501103

男性，60岁，因出现双眼视力模糊2个月余而来院就诊。


眼底镜检查显示双侧视网膜静脉扩张、迂曲以及视网膜出血（如图A和B分别显示右、左眼）。双眼最佳矫正视力为10 / 20。



荧光血管造影结果显示视网膜静脉串珠样图案和多出血管动脉瘤样病变（如图C和D）。

血清蛋白免疫固定法检测显示血清内IgM和κ轻链异常增多。

骨髓活检显示浆细胞样淋巴细胞浸润达10%-13%。

针对该患者的病情，主治医师对其进行七次血浆置换治疗。治疗后，双眼视力检测结果逐渐提高到20 / 20。

在随后的跟踪访问中，眼底镜检查发现该男子的双侧视网膜静脉异常情况有明显地好转。

巨球蛋白血症是一种源于能分化为成熟浆细胞的B淋巴细胞的恶性增生性疾病，该病有其独特的临床病理特点，主要表现为骨髓中有浆细胞样淋巴细胞浸润，并合成单克隆lgM。

此外，患有该病的患者一般会出现高粘滞综合征，它可以表现为患者视力下降、皮肤出血以及其他的一些神经症状等。但一般很少会出现类似于本案例中血管出现异常的症状。

原始出处：

Woojin Kim, M.D., and Euiyong Kweon, M.D., Hyperviscosity-Related Retinopathy in Waldenström’s Macroglobulinemia，N Engl J Med 2016;January 7, 2016


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    2016-01-22 xzw113

    #球蛋白#

    0

  2. [GetPortalCommentsPageByObjectIdResponse(id=2035122, encodeId=3b8e20351224b, content=<a href='/topic/show?id=5a426866eae' target=_blank style='color:#2F92EE;'>#球蛋白#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=21, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=68667, encryptionId=5a426866eae, topicName=球蛋白)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=2317301, createdName=xzw113, createdTime=Fri Jan 22 23:00:00 CST 2016, time=2016-01-22, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=57091, encodeId=20605e09148, content=再看一遍, beContent=null, objectType=article, channel=null, level=null, likeNumber=100, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160113/IMG569619FEB03536446.jpg, createdBy=85f81678980, createdName=Lynee劲飞扬, createdTime=Wed Jan 13 22:41:00 CST 2016, time=2016-01-13, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1269816, encodeId=d64b12698168c, content=<a href='/topic/show?id=979690e33bd' target=_blank style='color:#2F92EE;'>#视网膜#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=29, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=90733, encryptionId=979690e33bd, topicName=视网膜)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=742793, createdName=zutt, createdTime=Fri Jan 08 22:00:00 CST 2016, time=2016-01-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1413866, encodeId=2758141386647, content=<a href='/topic/show?id=27b290e82f4' target=_blank style='color:#2F92EE;'>#视网膜病变#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=90782, encryptionId=27b290e82f4, topicName=视网膜病变)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=f7143034078, createdName=zhanfl, createdTime=Fri Jan 08 22:00:00 CST 2016, time=2016-01-08, status=1, ipAttribution=)]
    2016-01-13 Lynee劲飞扬

    再看一遍

    0

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    2016-01-08 zutt

    #视网膜#

    0

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    2016-01-08 zhanfl

    #视网膜病变#

    0