m蛋白鉴别诊断-广州

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1、ၡᕡᙱዤየᨏ:Mᢓጮԏᰄڦ᦬ෙ๫ڻ۹Ղܐ޾܅ᴺᤅ႖ٖᑀlijian@pumch.cnԅMᢓጮҘᤅႴᢓጮኪ်ᤅ/ੳعዖࢴਧኪ်ᤅႴ჋ᐶ᫷᱾ྲ●ᤅႴ᯿᫷᱾(HLC)●԰ԅᤑش●ṋṚၞୗᕡᙱ༄ၥ●ํӞӻᴡ௔੪ݢզտ᦬෸̶̶̶Mᢓጮ+MMMGUSҘҘҘMᢓጮጱᰄڦ᦬ෙMᢓጮ+IgM+ᶋIgM+IgM-κ/λIgM-κIgM-λᶋIgM-κ/λᶋIgM-κᶋIgM-λWMMGUSALMGUSFanconiALٯٽᵞᔰየMMMMLCDDPOEMSᖓ஄Schnitzlerᖓ஄B-NHLB-NHLClarksonየᏝ௔ᔌ႖௔࿜ᙠIgM-PNٯቖᢓጮᤅዩTEMPICase●M/

2、52●༄ݎሿSPEP:Mᢓጮ3g/L●෫֎ᵋዩᇫҔ෬ஃྋଉ●ᤅIFE:IgA-k(+);ੳIFE(-);FLC-R=130/12.4●24hUP0.05g;cTnI/proBNP࣐ྋଉ;ALPྋଉ●BM:PC3%Ҕ●BMၞୗ:0.6%/300ӡӻᕡᙱ:CD138+CD38+CD56+●BM-FISH:1q2137%;13q-/17p-/IgH᯿ഭ࣐ᴢ௔1.఺Ԏӧกጱܔظᵇعዖቖᢓጮᤅዩ(MGUS)MGUSSMMMMMᢓጮ>30g/L̤̤BM-PC>10%̤̤SLimCRAB̤CRAB•PCीྠฎየࢩSLim•BMPC≥60%•C:ṛᰠᤅዩҔ>11mg/dL,ᴻ

3、क़ከෟԺ•Involved:uninvolved•R:ᙟۑᚆӧقҔeGFR<40ml/min౲Cr>177μmol/L;ᙟၚ༄ጱ᯿ᥝ௔serumfreelightchain•A:ᨹᤅҔ<100g/L౲ྋଉӥᴴ<20g/L;ᴻक़០ِ௔/ACD/MDSᒵratio≥100•B:ṋየҔvsṋᨶ዇ຂ/᫨ᑏጣ;MRI̵PET/CT;ṋդᨀຽᦕᇔ•>1focallesionsonMRIMGUSጱݎየሲ54.631>50y:1.32%3.75>70y:3.43%3.026(%)2.5ݎኞሲ1.382MGUS0.8941.250.47900.1060<3030-3940-49

4、50-5960-6970-79>80ଙἻ(઎)PUMCH༄Ոᗭහഝ(n=123400)MGUSጱᬰ઀ཛྷୗGenetichitMMᕡᙱीྠWM/LPLMGUSMGUSMᢓጮဃᑌALAlteredproteinLCDDMGUSMGUSጱᬰ઀ᷚᴾ:1%/ଙMGUSᬰ઀ጱܧᴾړ੶MGUSጱလዌ݊ᵋ᦬ܻڞړᕟचᕚᦧ֌ᵋ᦬ᦧ֌ᵋ᦬෸ᳵܧ•የݥ/໒༄ັ6mٖSPEPྯ2-3ଙ•IgG•CBC•FLCRྋଉ•Ca,Cr(eGFR)•M<15g/L•24hੳᢓጮӾ/ṛܧ•ṋṚၼᇆ+ၚ༄6mٖCBC+SPEPྯଙ1ེ•ᕡᙱ᭳փ਍+FISHᕣ᫝•ṋṒᦧհӾṛܧ,•NT-pro-BN

5、P•NT-pro-BNPྯଙ1ེ֎FLCR୑ଉ•ALP•24hੳᢓጮᕣ᫝•ALPCaseMGUS●M/52●༄ݎሿSPEP:Mᢓጮ3g/L●෫֎ᵋዩᇫҔ෬ஃྋଉӾܧMGUS֎FLCR୑ଉ●ᤅIFE:IgA-k(+);ੳIFE(-);FLC-R=130/12.4•6-12m॔ັӞེ●24hUP0.05g;cTnI/proBNP࣐ྋଉ;ALPྋଉ●BM:PC3%Ҕ•ᦄఝAL᫨۸●BMၞୗ:0.6%/300ӡӻᕡᙱ:CD138+CD38+CD56+●BM-FISH:1q2137%;13q-/17p-/IgH᯿ഭ࣐ᴢ௔2.ALࣳႚᔇ໏ݒ●错配后的反向β折叠蛋白沉积●刚

6、果红染色阳性●组织内的均质-无定型物质的沉积●大多数都为λ轻链型Ԇᥝᚍ࢏ݑᔴᇙᅩᚍ࢏ᤒሿ୽؟਍ਫḵਰஞᚍ●ᴴګ௔ஞᙄየ●EKGғᙏ੕ኪܴ●cTnI●ஞۑᚆᤗᒑ●UCGғஞਰॅ/ਰᳵᵍीܹ;ྷቄቼ●NT-proBNP໏ࢧ्;EFӥᴳᙟᚍ●ᢓጮੳ݌ᙟୠᄓ௔࣐۰ीय़●24hੳᢓጮ●ᙟۑᚆӧق●Ccrᙋᚍ●ᙋᯠ୑ଉᙋᚍୠᄓ௔ीय़ALP܋ṛ●ᙋय़PNࢥᙏἋ๙෫ٺVCN;ᴳӥPMC:EMGێౌAN௔ᤅܴᒈᤅܴ஽ወکႚᔇ໏ݒጱݢᚆ•ӧกܻࢩጱᢓጮੳ/ᙟየᖓ஄•ӧกܻࢩጱஞᙄየݒ/ஞۑᚆӧق•ӧกܻࢩጱᙋय़/ᙦᓕᯠीṛ•ӧกܻࢩጱޮࢱᐟᕪየݒ•ӧกܻࢩጱጼᙐڊᤅ•ӧกܻࢩጱᛢ

7、ᙑय़ܻݎ௔ALࣳႚᔇ໏ݒጱ᦬ෙၞᑕٌ՜ᔄࣳIFE/FLC੔ತMᢓጮIHC/IF/MSALَࣳࣳᕟᕢၚ༄ᔮᕹࣳvsੴᅏࣳԁଥᤒሿڟຎᕁວᜋੴᅏࣳԁଥMS᦬ෙṛଶወ᦬ALᶼړ๗:Mayo04/12ړ๗I๗cTnT(I)<0.035(0.1)ug/L;NT-proBNP<332ng/Lഝܧᴾࢩᔰහፓ(0~3ӻ)ړ౮1ӗ4๗II๗cTnT(I)≥0.035(0.1)ug/L;౲NT-proBNP≥332ng/L•pro-BNP>1800III๗cTnT(I)≥0.035(0.1)ug/L;޾NT-proBNP≥332ng/L•cTnT>0.025IIIa/b๗IIIa

8、:NT-proBNP<8500ng/L