Monoclonal gammopathy yog ib lub tswv yim sib sau ua ke uas muaj ntau yam kab mob, qhov tshwm sim ntawm qhov tshwm sim yog ua raws li kev ua txhaum ntawm kev ua haujlwm ntawm B-lymphocytes, ua rau cov kab mob tsis tu ncua ntawm ib qho clone ntawm immunoglobulins lossis lawv cov saw hlau.
zoo thiab phem yam ntawm tus kab mob
Yog li, monoclonal gammopathy tuaj yeem ua tau zoo thaum cov kab mob clonal ntawm cov hlwb uas tso tawm cov tshuaj tiv thaiv kab mob tsis zoo tsis zoo rau ntau thiab loj hlob uncontrollably los yog chaotically nce zus tau tej cov proteins txawv txav.
Pathology kuj tuaj yeem ua rau mob tsis zoo, thaum cov neeg clonal tsim cov protein ntau txawv txav yuav ua rau tsis muaj kev tswj hwm kev loj hlob tsis tu ncua, thiab tib lub sijhawm rau kev loj hlob. Raws li qhov tshwm sim, qhov tso tawm ntawm cov protein no feem ntau nce.
Cov ntawv phem ntawm monoclonal gammopathy suav nrog, piv txwv li, Waldenström's macroglobulinemia, kab mobteeb chains, thiab lwm yam. Daim ntawv malignant ntawm tus kab mob no feem ntau tsis tshua nkag siab rau cov tshuaj kho mob piv rau feem ntau lwm cov hemoblastoses.
ua rau ntawm pathology
Qhov tshwm sim ntawm tus kab mob no nce mus rau lub hnub nyoog (los ntawm ib feem ntawm cov neeg muaj hnub nyoog 25 mus rau plaub feem pua ntawm cov neeg tshaj xya caum). Monoclonal gammopathy tshwm sim nyob rau hauv ua ke nrog rau lwm yam pathologies nyob rau hauv uas cov zus tau tej cov M-protein yog tshwm sim los ntawm cov tshuaj tiv thaiv tsim nyob rau hauv loj qhov ntau nyob rau hauv teb rau ib tug ntev antigenic stimulus.
Nws yog ib qho tsim nyog sau cia tias cov ntaub ntawv benign ntawm monoclonal gammopathy feem ntau txhim kho raws li qhov tshwm sim ntawm tus mob overstimulation. Qhov kawg tsuas yog ua rau muaj kev loj hlob ntawm qee qhov clones ntawm plasma hlwb. Piv txwv li, qhov no yog qhov xwm txheej ntawm benign gammopathy, uas tau pom tom qab hloov pob txha pob txha. Ntau zaus tsawg dua, qhov no tau kho tam sim tom qab hloov cov ntaub so ntswg los yog muaj qee yam kab mob.
Symptoms
Monoclonal gammopathy feem ntau asymptomatic hauv tib neeg, txawm hais tias peripheral neuropathies tuaj yeem tshwm sim. Txawm hais tias muaj tseeb tias feem ntau cov piv txwv ntawm tus kab mob no tsis zoo, hauv 25 feem pua ntawm cov neeg mob tus kab mob no tuaj yeem mus rau B-cell hlav, thiab ntxiv rau, rau myeloma lossis macroglobulinemia.
Cov tsos mob ntawm monoclonal gammopathy yuav tsum tsis txhob ignored.
Diagnosis
Cov ntsiab lus ntawm immunoglobulin hauv cov ntshav yog ntsuas los ntawm electrophoresis. Immunoglobulins tuaj yeem txav mus rau hauv qhov hluav taws xob ntawm qhov sib txawv ntawm qhov nrawm thiab tsim lub ncov dav dav hauv thaj chaw gamma globulin. Nrog rau txoj kev loj hlob ntawm monoclonal gammopathy (ICD-10 code - D47.2), cov ntsiab lus ntawm gamma globulins nyob rau hauv cov ntshav feem ntau nce nyob rau hauv tib neeg, thiab ib tug ntse ncov yog pom nyob rau hauv lub electropherogram nyob rau hauv cheeb tsam no, uas yog hu ua M-gradient. Tsawg dua, nws yuav tshwm sim hauv thaj tsam ntawm beta lossis alpha globulins. Qhov rhiab heev pib ntawm txoj kev no yog tsib grams ib liter. Daim ntawv monoclonal ntawm M-gradient tau lees paub los ntawm kev tshawb pom ntawm ib hom hnyav thiab lub teeb chains thaum immunoelectrophoresis. Yog li, M-gradient raug ntsuas ntau (siv electrophoresis) thiab zoo (siv immunoelectrophoresis).
Yog tias qhov xwm txheej monoclonal ntawm kev zais cia tau raug pov thawj, ces nws yog qhov tsim nyog siv electrophoresis yav tom ntej. Tus nqi ntawm M-gradient qhia qhov loj ntawm cov qog. M-gradient yog ib qho kev txhim khu kev qha tab sis cov qog nqaij hlav tshwj xeeb. Nws tshwm sim tsis tau tsuas yog nyob rau hauv monoclonal gammopathy, tab sis kuj nyob rau hauv ntau yam lymphoproliferative pathologies. Piv txwv li, qhov no tau pom thaum tus neeg mob muaj mob lymphocytic leukemia thiab cellular lymphoma. Nws tseem raug kho rau keeb kwm yav dhau los ntawm malignant neoplasms nyob rau hauv daim ntawv ntawm mob myeloid leukemia, mob cancer ntawm lub mis thiab txoj hnyuv, thiab nyob rau hauv tas li ntawd, tiv thaiv keeb kwm ntawm ib tug xov tooj ntawm autoimmune pathologies (rheumatoid mob caj dab, myasthenia gravis, autoimmune hemolytic anemia nrog txias.tshuaj tiv thaiv). Thiab nrog rau lwm yam kab mob (cirrhosis ntawm daim siab, sarcoidosis, kab mob cab, pyoderma gangrenosum, Gaucher tus kab mob).
Ib tus kab mob tsis tshua muaj hu ua Gotgron's scleromyxedema feem ntau yog nrog los ntawm monoclonal gammopathy. Nrog rau tus kab mob no, qhov zoo tshaj plaws immunoglobulin, uas nqa cov saw hlau lambda, tuaj yeem tso rau hauv dermis. Nws tsis raug cais tawm tias cov tshuaj tiv thaiv no tuaj yeem raug coj tawm tsam qee cov ntsiab lus ntawm dermis.
Qhov xwm txheej ntawm M-gradient hauv kev sib txawv gammopathy tsis zoo ib yam. Nws sawv cev rau ib txwm immunoglobulins, txawv txav los yog lawv cov khoom tawg. Kev zais ntawm tus kheej chains tsis suav nrog: lub teeb lossis hnyav. Hauv nees nkaum feem pua ntawm cov neeg mob myeloma, tsuas yog lub teeb sawb tau zais, uas tshwm sim hauv cov zis hauv daim ntawv ntawm Jones protein. Qee cov qog ntshav hauv cov qog ntshav (tshwj xeeb yog cov pob txha thiab cov nqaij mos plasmacytomas) tso tawm cov protein monoclonal tsawg dua li ib feem peb ntawm txhua kis.
Thaum tus txheej txheem kuaj mob, cov kws kho mob nco ntsoov tias qhov zaus ntawm kev tso tawm ntawm immunoglobulins nyob rau hauv muaj ntau yam myeloma yog proportional rau lawv cov ntsiab lus ib txwm nyob rau hauv cov ntshav.
Kev kho mob
Kev kho rau monoclonal gammopathy (ICD-10 kab mob code D47.2) feem ntau tsis tas yuav tsum tau, txawm li cas los xij, vim tias muaj kev pheej hmoo ntawm hemoblastoses, cov neeg mob uas muaj tus kab mob paraproteinemia no yuav tsum tau kuaj xyuas tsis tu ncua. Hauv 47 feem pua ntawm cov neeg mob, paraproteinemia tuaj yeem nyob mus ib txhis (tab sis tsis ua rau tuag). Ntawm kaum rau feem pua ntawm cov neeg mobmyeloma tsim, thiab kaum feem pua ntawm cov qib ntawm paraprotein tuaj yeem nce mus rau qhov tseem ceeb tshaj peb grams.
Nws tseem tsim nyog sau cia tias peb feem pua ntawm cov neeg mob tsim thawj amyloidosis nrog rau Waldenström's macroglobulinemia, thiab lwm yam hemoblastoses tau pom nyob rau tib tus neeg mob. Hemoblastoses tuaj yeem tshwm sim hauv kaum xya feem pua ntawm cov neeg mob kaum xyoo tom qab. Lawv loj hlob nyob rau hauv 33 feem pua nees nkaum xyoo tam sim ntawd tom qab kuaj pom muaj tus kab mob benign monoclonal gammopathy. Qee lub sij hawm cov neeg mob tau kho nrog kws khomob.
Yog li, thawj qhov benign, mob ntev thiab asymptomatic tsis xav tau kev kho mob tshwj xeeb rau monoclonal gammopathy. Tab sis cov neeg mob xav tau kev saib xyuas ntev ntev ntawm cov tshuaj monoclonal immunoglobulin hauv cov ntshav. Qhov ntawd yog, txhua yam kev kho mob hauv qhov no los ntawm kev tos thiab saib xyuas.
Monoclonal gammopathy ntawm tsis paub keeb kwm
Qhov gammopathy no feem ntau tshwm sim los ntawm cov qog nqaij hlav ntawm cov ntshav. Cov hauv qab no yog cov yam ntxwv ntawm hom kab mob no:
- Nrog qhov tsis paub tseeb, tus neeg mob yuav muaj tus mob benign uas yuav ua rau precancerous.
- Kev pheej hmoo ntawm kev hloov pauv ntawm cov kab mob mus rau hauv cov qog yog nyob ntawm cov khoom ntawm cov nyiaj thiab hom paraprotein tsim, thiab ntxiv rau, ntawm qhov piv ntawm cov saw hnyav thiab lub teeb.
- Tus mob tuaj yeem tshwm sim los ntawm kev hloov pauv hauv B-lymphocytes, uas yog cov hlwb uas ib txwm tsim cov tshuaj immunoglobulins, uas yog, cov proteins.uas tiv thaiv kab mob.
- Feem ntau, tom qab nees nkaum xyoo, gammopathy ntawm qhov tsis paub keeb kwm tuaj yeem hloov mus ua qog rau plaub caug feem pua ntawm cov neeg mob.
- hom gammopathy no yeej tsis kho nrog kws khomob.
Cov yam ntxwv thiab cov yam ntxwv ntawm gammopathy, yam ntxwv los ntawm qhov tsis paub meej
Yog li, peb tab tom tham txog lub xeev biochemical thaum muaj qhov txawv txav M-protein hauv cov ntshav, uas yog ib qho tsis raug ntawm immunoglobulin lossis nws cov saw teeb, tsim los ntawm ib kab ntawm plasmacytic hlwb hauv cov pob txha pob txha. Nws yog hu ua tsis raug, vim nws tsis ua ib qho haujlwm tseem ceeb rau lub cev, ua raws li kev sib yuav hauv kev tsim cov protein. Hauv lub xeev ib txwm muaj, nws cov concentration hauv cov ntshav yuav tsum tsawg.
Feem ntau, M-protein qib qis thiab tsis nce ntxiv raws sijhawm. Tab sis nyob rau qee qhov xwm txheej, nws tuaj yeem ua rau muaj kev cuam tshuam loj heev, tig mus rau ntau yam myeloma lossis lwm yam kab mob monoclonal gammopathy.
Pathology, uas yog tus cwj pwm los ntawm qhov tsis paub tseeb, yog tus cwj pwm los ntawm kev faib qeeb ntawm cov plasmacyte clone thiab tso tawm ntawm M-protein hauv tus nqi qis. Tsis tas li ntawd, tawm tsam keeb kwm ntawm tus kab mob no, tsis muaj cov tsos mob ntawm ntau yam myeloma, AL-amyloidosis lossis lwm yam kab mob lymphoproliferative.
Qhov zaus ntawm cov kab mob no hauv cov pej xeem yog kwv yees li ib feem pua thiab nce, raws li txoj cai, nrog rau hnub nyoog. Tom qab tsib caug xyoo, tus kab mob no muaj nyob rau hauv peb feem pua ntawm cov pejxeem, thiab ntawm cov txiv neej uas muaj hnub nyoog yim caum, txhua kaum ob. Monoclonal gammopathy ntawm qhov tsis tseem ceeb ntawm cov nyiaj rau rau caum feem pua ntawm tag nrho cov pathologies ntawm hom no.
Yog vim li cas
Qhov ua rau ntawm daim ntawv no ntawm gammopathy, raws li lub npe qhia, hmoov tsis meej. Tsuas yog cov kev pheej hmoo hauv qab no paub:
- Thawj zaug, qhov no yog hnub nyoog, txij li nws laus dua, qhov kev pheej hmoo siab dua.
- Tus kab mob no feem ntau cuam tshuam rau txiv neej.
- Sib cuag nrog tshuaj tua kab, txhais tau tias cov neeg ua liaj ua teb muaj kev pheej hmoo tshwj xeeb.
- Kev muaj ntau yam myeloma lossis monoclonal gammopathy ntawm qhov tsis paub keeb kwm ntawm cov txheeb ze thiab ncaj qha.
Tam sim no, nws tsis yog raws nraim tsim, raws li qhov tshwm sim ntawm cov qog nqaij hlav. Muaj ib qho kev xav ua haujlwm txog kev hloov pauv hauv cov noob caj noob ces code rau kev tsim ntawm immunoglobulin thiab cov tsos ntawm M protein. Ncaj nraim ntawm monoclonal gammopathy thiab ntau yam myeloma lies ib txoj hauv kev ntev ntawm kev hloov kho caj ces hauv cov ntshav plasma. Pathology nrog cov keeb kwm tsis meej qhia ib kab ntawm B-lymphocytes uas tsim M-protein ntau dua. Ntau myeloma yog hu ua clone ntawm cov qog hlwb, uas yog thawj zaug hauv cov pob txha, thiab tom qab ntawd kis mus rau lwm yam kabmob.
YKev faib tawm ntawm pathology
Monoclonal gammopathy ntawm tsis paub keeb kwm hauv cov tshuaj tau muab faib ua cov kev hloov pauv hauv qab no:
- Lymphoplasmacytic gammopathy, uas tsis tshua muaj peev xwm tig mus rauNtau yam myeloma, tab sis tuaj yeem hloov mus ua macroglobulinemia lossis lwm yam tsis yog Hodgkin's lymphomas.
- Plasmacytic gammopathy, uas yog ib qho kev loj hlob ntawm cov plasma hlwb.
Diagnosis
Raws li ib feem ntawm txoj kev tshawb fawb rau monoclonal gammopathy ntawm MGUS tsis paub meej thiab kev kuaj sim, cov neeg mob yuav tsum tau ua raws li cov txheej txheem hauv qab no:
- Kev kuaj ntshav kom kuaj pom qis hemoglobin.
- Lub raum sampling. Cov neeg mob feem ntau raug kuaj rau creatinine los ntsuas lub raum ua haujlwm.
- Kev tshawb fawb ntawm cov kab mob ntshav, uas yog, calcium yog tshuaj xyuas, uas yog qhov qhia txog kev puas tsuaj ntawm cov pob txha.
- Kev ua cov ntshav protein electrophoresis nrog rau cov protein immunofixation.
- Kev txiav txim siab ntawm immunoglobulins hauv cov ntshav.
- Tsim kom muaj lub teeb ci dawb hauv cov ntshav.
- Kev txiav txim siab ntawm cov beta-2-microglobulin.
Instrumental exams
Raws li kev tshawb fawb, kev saib xyuas yog them rau cov txheej txheem hauv qab no:
- X-ray ntawm cov pob txha pob txha, yog li, kev kawm ntawm tus txha nraub qaum, pob txha taub hau, pob txha hauv plab, lub duav thiab lub hauv caug pob qij txha thiab lwm yam.
- Kev ua tau zoo ntawm kev kuaj magnetic resonance, uas yog tus qauv kub hauv kev kuaj mob ntawm cov pob txha hloov hauv monoclonal gammopathy. Nws yog ib nqi sau cia hais tias yog hais tias mob tshwm sim nyob rau hauv ib tug ntawm cov pob txha, magnetic resonance imaging yog yuav tsum tau.
- Computed tomography ua ntejcov kab yog tsom rau kev tshawb pom ntxov ntawm lwm cov qog ntshav uas tus kab mob no tuaj yeem ua tiav.
- Ua ib qho sternal puncture lossis trephine biopsy. Cov txheej txheem no tsim nyog yog tias koj muaj qhov txawv txav kappa-lambda index lossis ib qho ntawm cov tsos mob.
- Kev ua densitometry, uas yog, ntsuas cov pob txha ntom.
monoclonal gammopathy ntawm lub hauv paus tsis paub kho tau li cas? Qhov no pathology tsis xav tau kev kho mob. Txawm li cas los xij, qee zaum lub xeev ntawm "kev noj qab haus huv tsis tiav" hloov mus ua tus kab mob hnyav dua, yog li koj yuav tsum tau saib xyuas tsis tu ncua los ntawm hematologist. Nws yuav siv sij hawm ntau xyoo ntawm kev soj ntsuam ua ntej qhov kev hloov pauv no tshwm sim.
Nyob rau me nyuam
Cov kab mob hauv nqe lus nug yog ib qho kab mob hauv zos thiab feem ntau txwv tus kheej hauv cov menyuam yaus hnub nyoog nruab nrab ntawm tsib thiab kaum thiab cov tub ntxhais hluas txog peb caug. Feem ntau cov kab mob cuam tshuam hauv monoclonal gammopathy hauv cov menyuam yaus yog cov pob txha, thiab ntxiv rau, lub ntsws. Kev koom tes ntawm pob txha feem ntau tshwm sim, tab sis kev koom tes ntawm cov qog endocrine feem ntau pom. Tus kab mob no tsis zoo rau cov menyuam yaus hnub nyoog qis dua ob xyoos.
Pab mob plab
Mob nraub qaum nrog monoclonal gammopathy feem ntau tshwm sim hauv cov neeg mob uas muaj cov qog loj hlob nyob rau hauv vertebra lossis hauv thaj tsam ntawm cov nqaij mos paravertebral. Qhov tsis xis nyob zoo li no tuaj yeem tshwm sim thaum muaj cov tsos mob ntawm compression ntawm cov hauv paus hniav lossis txha caj qaum. Raws li ib feem ntawm kev kawm rauTxhawm rau tshem tawm qhov tsis xis nyob, cov neeg mob tau muab cov duab sib nqus resonance, uas tso cai rau kom pom kev puas tsuaj rau tus txha caj qaum, thiab ntxiv rau, txhawm rau txheeb xyuas qhov mob hnyav ntawm tus txha caj qaum.
Kev tshuaj xyuas cov kws kho mob thiab cov neeg mob
Nyob rau hauv kev tshuaj xyuas txog kev txhawb nqa rau cov neeg mob uas muaj tus kab mob no, cov kws kho mob sau tias cov protein feem electrophoresis yog ib qho cuab yeej tseem ceeb rau kev tshuaj xyuas thiab kuaj cov kab mob malignant ntawm monoclonal gammopathy cuam tshuam nrog cov kab mob immunoproliferative.
Nyob rau hauv cov lus ntawm cov neeg mob txog tus kab mob no, nws tau sau tseg tias nws yog ib qho tseem ceeb heev uas yuav tsum tau ua tiav nws qhov kev kuaj mob ntxov thiab kev tiv thaiv. Cov kws tshaj lij tau hais tias kev siv dav dav ntawm kev sim tshuaj biochemical thiab ntshav electrophoresis tau ua rau muaj peev xwm kuaj pom tus kab mob sai sai.
Koj puas tuaj yeem nyob nrog monoclonal gammopathy? Raws li cov neeg mob, yog tias ib tug neeg tau kuaj pom tus kab mob no, nrog rau tom qab lees paub qhov kev kuaj mob no raws li cov kev xav tau niaj hnub no, nws raug nquahu kom ua raws li qee qhov algorithm rau kev saib xyuas tus kws kho mob tshwj xeeb. Yog tias tus neeg mob tsis muaj kev tsis txaus siab nyob rau thawj xyoo, kev tshawb fawb ntawm cov tshuaj paraprotein tau ua, uas yuav tsum tau ua txhua peb lub hlis. Kev tshawb fawb magnetic resonance, raws li cov neeg mob, yog ua txhua txhua rau lub hlis. Yog tias tsis muaj qhov nce ntxiv hauv paraprotein, tom qab ntawd cov kev tshawb fawb ntxiv tau ua txhua txhua kaum ob lub hlis.
Yog li, monoclonal gammopathy (thiab lawvKuj hu ua immunoglobulinopathies lossis paraproteinemia) yog ib hom kab mob sib txawv ntawm cov kab mob uas tshwm sim los ntawm kev loj hlob ntawm cov qog ntshav lymphoid uas tso tawm cov tshuaj immunoglobulins. Lub ntsiab qhov txawv ntawm cov kab mob no yog kev tsim cov monoclonal immunoglobulin, uas tau txiav txim siab hauv cov ntshav ntshav lossis zis.
