Monoclonal gammopathy (GM) tau txhais los ntawm qhov muaj nyob hauv cov ntshav thiab / lossis cov zis ntawm monoclonal immunoglobulin. Nws tuaj yeem cuam tshuam nrog hemopathy tsis zoo, txwv tsis pub nws raug hu ua monoclonal gammopathy ntawm qhov tsis tau txiav txim siab tseem ceeb (GMSI).

Txog kev kuaj mob, kuaj kab mob microbiological ua rau nws muaj peev xwm txheeb xyuas monoclonal immunoglobulin ntau dhau. Kev kuaj mob, tshuaj lom neeg thiab duab hluav taws xob tuaj yeem taw qhia rau hemopathy qhov kev kuaj mob ntawm GMSI yog kev kuaj mob sib txawv.

Lus txhais

Monoclonal gammopathy (GM) tau txhais los ntawm qhov muaj nyob hauv cov ntshav thiab / lossis cov zis ntawm monoclonal immunoglobulin. Immunoglobulins yog cov protein hauv tib neeg cov ntshav uas muaj lub zog tiv thaiv kab mob. Lawv tau sib sau ua ke hauv cov ntshav ntshav, cov cell ntawm cov qog ua kua hauv lub cev thiab cov qog ntshav. Yog li GM ua tim khawv rau kev nthuav dav ntawm clone ntawm cov ntshav ntshav tsim cov monoclonal immunoglobulin.

hom

GMs tuaj yeem faib ua 2 pawg:

• Monoclonal gammopathies cuam tshuam nrog hematologic malignancies
• Monoclonal gammopathies ntawm qhov tsis tseem ceeb (GMSI)

Ua rau

Rau monoclonal gammopathies cuam tshuam nrog hemopathies phem, lub hauv paus tseem ceeb yog:

• Ntau yam myeloma: qog ntawm cov pob txha pob txha tsim los ntawm kev nthuav dav ntawm cov ntshav ntshav txawv txav
• Macroglobulinemia (Waldenström tus kab mob): muaj qhov txawv txav hauv cov ntshav macroglobulin
• B qog lymphoma

GMSI tuaj yeem cuam tshuam nrog ntau yam kab mob tsis zoo:

• Cov kab mob autoimmune (rheumatoid polyathritis, Sjögren's syndrome, mob lupus)
• Kab mob sib kis (mononucleosis, mob qhua pias, HIV, kab mob siab C)
• Kab mob sib kis (endocarditis, osteomyelitis, tuberculosis)
• Kab mob sib kis (leishmaniasis, malaria, toxoplasmosis)
• Cov kab mob ntev xws li mob cholecystitis ntev (mob ntawm lub zais zis)
• Ntau yam mob xws li tsev neeg hypercholesterolemia, Gaucher tus kab mob, Kaposi's sarcoma, lichen, kab mob hauv siab, myasthenia gravis (teeb meem ntawm kev xa cov hlab ntsha ntawm cov hlab ntsha mus rau cov leeg), ntshav tsis txaus lossis thyrotoxicosis

diagnostic

GM feem ntau pom muaj xwm txheej, thaum kuaj sim ua rau lwm yam.

Txhawm rau txheeb xyuas tus neeg sawv cev monoclonal ntau dhau, qhov kev ntsuas muaj txiaj ntsig tshaj plaws yog:

• Electrophoresis ntawm cov ntshav cov ntshav: cov txheej txheem tso cai txheeb xyuas thiab cais cov protein ntawm cov ntshav hauv qab qhov kev txiav txim ntawm hluav taws xob
• Immunofixation: txheej txheem tso cai kuaj pom thiab ntaus ntawv monoclonal immunoglobulins
• Kev ntsuam xyuas immunoglobulin: txheej txheem uas cais cov protein los ntawm cov ntshav thiab txheeb xyuas lawv raws qhov pom ntawm cov tshuaj tiv thaiv kab mob uas lawv tsim

Tom qab ntawd qhov kev kuaj mob mus dhau los nrhiav qhov ua rau GM. Ntau qhov chaw kho mob, tshuaj lom neeg lossis tshuaj tua hluav taws xob yuav tsum qhia ntau yam mob myeloma:

• Kev poob phaus, mob pob txha mob, mob pob txha puas
• Anemia, hypercalcemia, lub raum tsis ua haujlwm

Lwm qhov tshwm sim tam sim taw tes rau hemopathy:

• Lymphadenopathy, splenomegaly
• Qhov txawv txav hauv cov ntshav suav nrog: ntshav tsis txaus, thrombocytopenia, lymphocytosis ntau dhau
• Syndrome d'hyperviscosité

GMSI tau txhais tias yog GM yam tsis muaj kev kuaj mob lossis kuaj pom ntawm hematologic malignancy. Hauv kev kho mob niaj hnub, qhov no yog kev kuaj mob ntawm kev cais tawm. Cov txheej txheem siv los txheeb xyuas GMSI yog:

• Monoclonal tivthaiv tus nqi <30 g / l 
• Kev txheeb ze ruaj khov nyob rau lub sijhawm ntawm cov khoom siv monoclonal 
• Cov qib ntshav qub ntawm lwm yam immunoglobulins
• Tsis muaj kev puas tsuaj ntawm cov pob txha, ntshav tsis txaus thiab lub raum tsis zoo

Qhov tshwm sim ntawm GMSI nce nrog hnub nyoog los ntawm 1% ntawm 25 xyoo mus rau ntau dua 5% dhau 70 xyoo.

GMSI feem ntau yog asymptomatic. Txawm li cas los xij, monoclonal antibody tuaj yeem khi rau qab haus huv thiab ua rau loog, tingling, thiab tsis muaj zog. Cov neeg uas muaj tus mob no feem ntau yuav muaj kev puas tsuaj ntawm cov nqaij pob txha thiab pob txha.

Thaum GM cuam tshuam nrog lwm tus kab mob, cov tsos mob yog cov kab mob.

Ntxiv mus, monoclonal immunoglobulins tuaj yeem ua rau muaj teeb meem tsawg:

• Amyloidosis: tso nyiaj ib feem ntawm cov protein monoclonal hauv cov kabmob sib txawv (ob lub raum, lub plawv, qab haus huv, lub siab) uas tuaj yeem ua rau lub cev tsis ua haujlwm.
• Plasma hyperviscosity syndrome: nws yog lub luag haujlwm rau qhov muag tsis pom kev, cov tsos mob ntawm lub paj hlwb (mob taub hau, kiv taub hau, nkees nkees, kev saib tsis taus) thiab cov tsos mob hemorrhagic
• Cryoglobulinemia: kab mob tshwm sim los ntawm kev muaj cov ntshav ntawm immunoglobulins uas nag lossis daus thaum kub qis dua 37 °. Lawv tuaj yeem ua rau pom ntawm daim tawv nqaij (purpura, Raynaud qhov tshwm sim, qhov qis necrosis), polyarthralgia, neuritis thiab glomerular nephropathies.

Rau IMGs, tsis pom zoo kho mob. Cov kev tshawb fawb tsis ntev los no qhia tias IMGTs nrog cov pob txha poob qis tuaj yeem tau txais txiaj ntsig los ntawm kev kho nrog bisphosphonates. Txhua txhua 6 txog 12 lub hlis, cov neeg mob yuav tsum tau mus kuaj mob thiab ua cov ntshav thiab cov zis protein electrophoresis txhawm rau ntsuas kev mob zuj zus.

Hauv lwm qhov xwm txheej, kev kho yog qhov ua rau.

Hauv ib feem ntawm qhov nce txog 25% ntawm cov neeg mob, kev hloov pauv ntawm GMSI mus rau cov kab mob phem hematologic tau pom. Cov neeg uas muaj GMSI tau ua raws lub cev, ntshav thiab qee zaum tso zis kuaj ob zaug hauv ib xyoos los tshuaj xyuas qhov ua tau kom muaj mob qog noj ntshav. Yog tias muaj kev tshawb pom pom ntxov, cov tsos mob thiab teeb meem tuaj yeem tiv thaiv lossis kho ua ntej.