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多发性骨髓瘤罕见并发症一例

2018-05-03 熊长明 肺血管病

患者,男,37岁。主因“活动后憋气1年余,鼻出血2月余。”曾于2008年4月就诊于北京协和医院。该患者于2006年下半年开始于饮酒﹑感冒﹑劳累后出现胸闷﹑憋气,无心前区疼痛,无咳嗽﹑咳痰﹑无晕厥。

临床资料】

患者,男,37岁。主因“活动后憋气1年余,鼻出血2月余。”曾于2008年4月就诊于北京协和医院。该患者于2006年下半年开始于饮酒﹑感冒﹑劳累后出现胸闷﹑憋气,无心前区疼痛,无咳嗽﹑咳痰﹑无晕厥。且易感冒﹑发烧,经常出现口周疱疹。2007年下半年开始于晨跑过程中有胸闷﹑憋气发作,减慢速度可缓解。曾在1次晨跑时出现一过性黑蒙,无意识丧失。2008年1月10日患者再次出现发热,Tmax 38.0℃,伴咳嗽﹑咳白痰﹑鼻出血 5~6次,自行处理后出血均可停止。曾于1次大量饮酒后咯血,共3~5口。2008年1月19日又出现鼻出血,量较大,处理后不能停止,遂至当地医院治疗,给予局部鼻孔填塞后出血停止。查血常规:PLT 53×109/L(余不详);超声心动图:右心增大,肺动脉压力102mmHg,心包少量积液;胸片:双肺纹理增多﹑增粗模糊,肺门影扩大﹑增浓;心影横径增大,右下肺动脉增宽;肺部增强CT:双肺平扫及肺动脉增强未见明显异常,心包积液。未予特殊治疗。

2008年3月20日至阜外医院住院,查血常规:PLT(33~57)×109/L,WBC﹑RBC基本正常;尿常规:正常;肝肾功能未见异常;补体:CH50 20.3U/ L,C3 50.8mg/dl,C4 10.6mg/dl;免疫球蛋白:IgG 56.9g/L,IgA 0.691g/L,IgM 0.276g/L;ESR 41mm/h;CRP19.5mg/L;ANA﹑抗ds-DNA﹑ACA﹑抗ENA﹑ACL﹑LA﹑ANCA﹑自身抗体﹑RF均阴性;甲状腺功能正常;血气分析:pH 7.415,PO2 80.2mmHg,PCO2 32.2mmHg,SpO2 96.5%;心电图:窦性心律,右心肥厚,ST-T改变;胸片:肺动脉高压;肺灌注显像:未见明显异常;超声心动图:估测肺动脉压力75mmHg,三尖瓣少~中量反流,肺动脉瓣少量反流;静态心肌代谢显像:右心室心腔扩大,代谢增强,余未见明显异常;右心导管检查示:重度肺动脉高压,平均肺动脉压90mmHg,吸入伊洛前列腺素后肺动脉压无明显下降;肺动脉造影示:主肺动脉及左右肺动脉扩张,肺动脉及其分支充盈尚可,未见狭窄﹑缺支及充盈缺损。诊断为“肺动脉高压,结缔组织病继发性肺动脉高压可能性大”,为进一步明确原发病2008年4月4日就诊于北京协和医院。

既往16岁左右曾患急性甲型肝炎,治疗后好转。饮酒20余年。家族史无特殊。

入院时查体:T 36.6℃,BP 90/60mmHg,右侧耳后﹑背部可见数个直径0.5~1.0cm的圆形皮疹,突出皮面,压之可褪色,无脱屑,右侧颈后﹑左侧颈后各可扪及一蚕豆大小淋巴结,质软﹑边界清楚,活动度可,无压痛,余浅表淋巴结未触及。双肺呼吸音清,未闻及干湿啰音,HR 100次/分,律齐,P2>A2,肺动脉听诊区第2心音分裂,心尖部可闻及舒张期杂音,腹软,无压痛,肝脾肋下未及,肠鸣音活跃,各关节无红肿,无压痛,活动无障碍,无杵状指,四肢肌力﹑肌张力正常,双下肢无水肿。

诊治经过:入院后完善检查:血常规WBC﹑RBC﹑HGB 正常,PLT 84×109/L。肝肾功能未见异常。凝血功能:PT 17.3秒,PT%57.9%,INR 1.43,APTT 52.9s,APTT-R 52.9秒。大便潜血:(-);尿常规:SG≥1.030,红细胞微量。1∶1正浆纠正试验(+);尿蛋白电泳:Alb%41.4%,α1 4.2%,α2 5.4%,β1 3.5%,β2 1.5%,γ 44%,A/G 0.71,M蛋白可见。血沉 81mm/h,CRP14.9mg/L,补体 CH50 25.8U/ml,C3 54.7mg/dl,C4 10.7mg/dl,免疫球蛋白:IgG 49.4g/L,IgA 0.682g/L,IgM 0.25lg/L。骨盆+颅骨:骨盆对称,两侧髋关节及股骨头形态尚可,两侧骶髂关节间隙尚可。S1隐裂。颅骨顶骨后部可见低密度影,余(-)。胸片:两肺纹理增多,边缘较清晰,双肺门影浓密,心缘饱满,心影肺动脉段略凸出。骨扫描:双肩﹑髋关节及双侧股骨下端﹑双胫骨上端放射性摄取异常增高,性质待定。余骨骼未见异常。骨穿检查结果:骨髓瘤细胞35%。治疗上继续给予地高辛0.125mg Qd,氯化钾缓释片 0.5g tid,呋塞米片 20mg Qd。目前多发性骨髓瘤诊断明确,分型为IgGκ型。目前血清钙和肾功能正常,IgG最高>50g/L,骨X线未见明确溶骨性破坏,可分为ⅡA期。转入血液科继续治疗。2008年4月16起予VAD+T方案化疗:长春地辛1mg D1~4,表柔比星 30mg D1~4,地塞米松40 mg D1~4,并予沙利度胺100mg qn,化疗过程顺利,一般情况好转出院。

出院诊断:多发性骨髓瘤,肺动脉高压。

【讨论】

肺动脉高压已作为多发性骨髓瘤的一种罕见并发症。特别是在POEMS综合征患者的诊断时常被提及。多发性骨髓瘤的患者可以胸闷﹑憋气﹑腹胀为首发表现,查体可有呼吸急促,心动过速,收缩早期奔马律,颈内静脉怒张,右侧胸腔积液,肝脾肿大伴腹水。血气分析可以提示低氧血症。首先在行超声心动图时可发现右心扩大,三尖瓣反流,肺动脉高压,室间隔矛盾运动,而左心室大小可以正常。心肌活检提示淀粉样变,随后再行血液学检查证实为多发性骨髓瘤。这些患者往往没有淀粉样变的其他临床特征。有文献报道,该类患者肺动脉高压的发生可能是由于淀粉样蛋白沉积于肺动脉所致,这种情况多见于AL淀粉样变性。也有报道认为血管活性因子的异常释放造成了肺动脉高压。应用西地那非治疗可以得到血流动力学和症状上的改善。

与此同时,多发性骨髓瘤确诊后并得到治疗的患者中也有新出现肺动脉高压的情况,这可能与应用沙利度胺治疗有关。有报道,一位多发性骨髓瘤患者在接受沙利度胺治疗7个月后出现头晕﹑乏力﹑气短症状。超声心动图提示肺动脉高压,但没有心肌淀粉样变的表现。肺脏和心肌活检未能证明微栓塞形成或淀粉样蛋白沉积,病理表现与特发性肺动脉高压相同。停用沙利度胺后5个月肺动脉压力逐渐下降,但患者最终因为多发性骨髓瘤进展及心力衰竭而死亡。目前沙利度胺造成肺动脉高压的原因尚不明确,但可以确定的是由于对血管内皮细胞和肌肉细胞的不可逆的药物作用使得有些患者再停药后肺动脉压力仍不能恢复至正常,而且应用前列腺素类似物治疗效果也并不理想。该患者治疗前就已发生肺动脉高压,在应用沙利度胺治疗过程中需进一步观察肺动脉压力变化。

另外,也有报道在多发性骨髓瘤的患者中也可能合并其他病因所致的肺动脉高压,特别是肺脏及肺血管本身的病变,因此需要先行肺动脉CT﹑肺通气/灌注扫描除外肺血栓栓塞症,肺部HRCT除外肺实质及间质病变,右心导管检查除外肺静脉闭塞性疾病,必要时进行肺脏活检,以免延误诊断及治疗。

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    2018-07-07 jml2009
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    2018-06-19 丁鹏鹏
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    2018-05-05 syscxl
  4. [GetPortalCommentsPageByObjectIdResponse(id=1889893, encodeId=1e5f18898937b, content=<a href='/topic/show?id=760c430895a' target=_blank style='color:#2F92EE;'>#多发性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=89, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=43089, encryptionId=760c430895a, topicName=多发性)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=04d6114, createdName=jml2009, createdTime=Sat Jul 07 09:39:00 CST 2018, time=2018-07-07, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1757982, encodeId=e6ad1e57982c6, content=<a href='/topic/show?id=84ba49031f9' target=_blank style='color:#2F92EE;'>#并发#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=77, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=49031, encryptionId=84ba49031f9, topicName=并发)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=a27e37327108, createdName=丁鹏鹏, createdTime=Tue Jun 19 13:39:00 CST 2018, time=2018-06-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1467315, encodeId=11ec146e31589, content=<a href='/topic/show?id=ef32e9539e8' target=_blank style='color:#2F92EE;'>#罕见#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=61, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=79539, encryptionId=ef32e9539e8, topicName=罕见)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=29f36868190, createdName=syscxl, createdTime=Sat May 05 05:39:00 CST 2018, time=2018-05-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=311913, encodeId=d0d53119138c, content=太复杂了-学习无止境啊, beContent=null, objectType=article, channel=null, level=null, likeNumber=115, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://q.qlogo.cn/qqapp/1103841572/6AAE0B891900F16725A1E4746F5293BB/100, createdBy=26792053039, createdName=bobogemini6686, createdTime=Fri May 04 10:24:53 CST 2018, time=2018-05-04, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=311705, encodeId=5555311e052b, content=学习一下谢谢, beContent=null, objectType=article, channel=null, level=null, likeNumber=96, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mONcle9pic3zSn0GqHrsfLGSUSR8zwnWWLT4FMl5XH8S58HuZslVkLsAPbXDXaliagK7TOxQfmngKibG1yrDonepCLDkcxGk8hS/0, createdBy=b8282062892, createdName=changjiu, createdTime=Thu May 03 21:54:26 CST 2018, time=2018-05-03, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=311652, encodeId=e137311652a8, content=谢谢分享.学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=116, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/NUyjXTCJjo6Nx0VGA4QMDzOP8oAgnV4h51EjSfehJ9QxyiaSmw8PB56BhY8KXFSxHCibXrzcy4abrzSibXQPxJ9kzrLcJyhhB8a/0, createdBy=1d3b1672603, createdName=明月清辉, createdTime=Thu May 03 15:56:28 CST 2018, time=2018-05-03, status=1, ipAttribution=)]
    2018-05-04 bobogemini6686

    太复杂了-学习无止境啊

    0

  5. [GetPortalCommentsPageByObjectIdResponse(id=1889893, encodeId=1e5f18898937b, content=<a href='/topic/show?id=760c430895a' target=_blank style='color:#2F92EE;'>#多发性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=89, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=43089, encryptionId=760c430895a, topicName=多发性)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=04d6114, createdName=jml2009, createdTime=Sat Jul 07 09:39:00 CST 2018, time=2018-07-07, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1757982, encodeId=e6ad1e57982c6, content=<a href='/topic/show?id=84ba49031f9' target=_blank style='color:#2F92EE;'>#并发#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=77, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=49031, encryptionId=84ba49031f9, topicName=并发)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=a27e37327108, createdName=丁鹏鹏, createdTime=Tue Jun 19 13:39:00 CST 2018, time=2018-06-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1467315, encodeId=11ec146e31589, content=<a href='/topic/show?id=ef32e9539e8' target=_blank style='color:#2F92EE;'>#罕见#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=61, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=79539, encryptionId=ef32e9539e8, topicName=罕见)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=29f36868190, createdName=syscxl, createdTime=Sat May 05 05:39:00 CST 2018, time=2018-05-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=311913, encodeId=d0d53119138c, content=太复杂了-学习无止境啊, beContent=null, objectType=article, channel=null, level=null, likeNumber=115, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://q.qlogo.cn/qqapp/1103841572/6AAE0B891900F16725A1E4746F5293BB/100, createdBy=26792053039, createdName=bobogemini6686, createdTime=Fri May 04 10:24:53 CST 2018, time=2018-05-04, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=311705, encodeId=5555311e052b, content=学习一下谢谢, beContent=null, objectType=article, channel=null, level=null, likeNumber=96, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mONcle9pic3zSn0GqHrsfLGSUSR8zwnWWLT4FMl5XH8S58HuZslVkLsAPbXDXaliagK7TOxQfmngKibG1yrDonepCLDkcxGk8hS/0, createdBy=b8282062892, createdName=changjiu, createdTime=Thu May 03 21:54:26 CST 2018, time=2018-05-03, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=311652, encodeId=e137311652a8, content=谢谢分享.学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=116, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/NUyjXTCJjo6Nx0VGA4QMDzOP8oAgnV4h51EjSfehJ9QxyiaSmw8PB56BhY8KXFSxHCibXrzcy4abrzSibXQPxJ9kzrLcJyhhB8a/0, createdBy=1d3b1672603, createdName=明月清辉, createdTime=Thu May 03 15:56:28 CST 2018, time=2018-05-03, status=1, ipAttribution=)]
    2018-05-03 changjiu

    学习一下谢谢

    0

  6. [GetPortalCommentsPageByObjectIdResponse(id=1889893, encodeId=1e5f18898937b, content=<a href='/topic/show?id=760c430895a' target=_blank style='color:#2F92EE;'>#多发性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=89, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=43089, encryptionId=760c430895a, topicName=多发性)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=04d6114, createdName=jml2009, createdTime=Sat Jul 07 09:39:00 CST 2018, time=2018-07-07, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1757982, encodeId=e6ad1e57982c6, content=<a href='/topic/show?id=84ba49031f9' target=_blank style='color:#2F92EE;'>#并发#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=77, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=49031, encryptionId=84ba49031f9, topicName=并发)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=a27e37327108, createdName=丁鹏鹏, createdTime=Tue Jun 19 13:39:00 CST 2018, time=2018-06-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1467315, encodeId=11ec146e31589, content=<a href='/topic/show?id=ef32e9539e8' target=_blank style='color:#2F92EE;'>#罕见#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=61, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=79539, encryptionId=ef32e9539e8, topicName=罕见)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=29f36868190, createdName=syscxl, createdTime=Sat May 05 05:39:00 CST 2018, time=2018-05-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=311913, encodeId=d0d53119138c, content=太复杂了-学习无止境啊, beContent=null, objectType=article, channel=null, level=null, likeNumber=115, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://q.qlogo.cn/qqapp/1103841572/6AAE0B891900F16725A1E4746F5293BB/100, createdBy=26792053039, createdName=bobogemini6686, createdTime=Fri May 04 10:24:53 CST 2018, time=2018-05-04, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=311705, encodeId=5555311e052b, content=学习一下谢谢, beContent=null, objectType=article, channel=null, level=null, likeNumber=96, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mONcle9pic3zSn0GqHrsfLGSUSR8zwnWWLT4FMl5XH8S58HuZslVkLsAPbXDXaliagK7TOxQfmngKibG1yrDonepCLDkcxGk8hS/0, createdBy=b8282062892, createdName=changjiu, createdTime=Thu May 03 21:54:26 CST 2018, time=2018-05-03, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=311652, encodeId=e137311652a8, content=谢谢分享.学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=116, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/NUyjXTCJjo6Nx0VGA4QMDzOP8oAgnV4h51EjSfehJ9QxyiaSmw8PB56BhY8KXFSxHCibXrzcy4abrzSibXQPxJ9kzrLcJyhhB8a/0, createdBy=1d3b1672603, createdName=明月清辉, createdTime=Thu May 03 15:56:28 CST 2018, time=2018-05-03, status=1, ipAttribution=)]
    2018-05-03 明月清辉

    谢谢分享.学习了

    0

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