Monoclonal gammopathy (GM) e faʻamatalaina e ala i le i ai i totonu o le toto ma / poʻo le urine o se immunoglobulin monoclonal. E mafai ona fesoʻotaʻi ma se faʻamaʻi pipisi, a leai e taʻua o le monoclonal gammopathy o le taua tele (GMSI).

Mo le su'esu'ega, o su'esu'ega microbiological e mafai ai ona iloa le monoclonal immunoglobulin i le tele. O fa'aaliga fa'a-ma'i, fa'aolaola ma fa'alāleo e mafai ona fa'asino ile hemopatī ae ole su'esu'ega ole GMSI ole su'esu'ega 'ese'ese.

Faamatalaina

Monoclonal gammopathy (GM) e faʻamatalaina e ala i le i ai i totonu o le toto ma / poʻo le urine o se immunoglobulin monoclonal. Immunoglobulins o polotini i le plasma o le tagata e iai mea e puipuia ai. O loʻo faʻapipiʻiina i totonu o sela plasma, sela o le lymphoid system e fausia i totonu o le spleen ma lymph nodes. O le mea lea e molimau le GM i le faʻateleina o se clone o sela plasma e maua ai se immunoglobulin monoclonal.

ituaiga

GMs e mafai ona faʻavasegaina i 2 vaega:

• Monoclonal gammopathies e fesoʻotaʻi ma faʻamaʻi hematologic
• Monoclonal gammopathies o le taua tele (GMSI)

Mafuaaga

Mo gammopathies monoclonal e fesoʻotaʻi ma faʻamaʻi mamaʻi, o mafuaʻaga autu o:

• Multiple myeloma: tumo o le ga'o ponaivi e mafua mai i le fa'ateleina o sela fa'aletonu ole plasma
• Macroglobulinemia (Waldenström's faʻamaʻi): o loʻo iai i ni aofaʻi faʻaletonu i le plasma o le macroglobulin.
• B lymphoma

GMSI e mafai ona fesoʻotaʻi ma faʻamaʻi eseese e le faʻaleagaina:

• Fa'ama'i fa'ama'i (rheumatoid polyathritis, Sjögren's syndrome, systemic lupus)
• Fa'ama'i pipisi (mononucleosis, tanesusu, HIV, hepatitis C)
• Fa'ama'i pipisi (endocarditis, osteomyelitis, tuberculosis)
• Fa'ama'i pipisi (leishmaniasis, malaria, toxoplasmosis)
• Fa'ama'i fa'ama'i e pei o le cholecystitis fa'ama'i (mumu o le gallbladder)
• O isi tulaga e pei ole hypercholesterolemia aiga, ma'i Gaucher, sarcoma Kaposi, lichen, ma'i ate, myasthenia gravis (fa'aletonu o le felauaiga o le neura mai le neura i le maso), anemia poʻo le thyrotoxicosis.

Diagnostic

GM e masani ona iloa faʻafuaseʻi, i le taimi o suʻega suʻesuʻe na faia mo isi mafuaaga.

Ina ia iloa le soona monoclonal sui, o suʻega sili ona aoga o:

• Electrophoresis o serum proteins: o se metotia e mafai ai ona iloa ma vavaeeseina le polotini o le serum i lalo o le gaioiga a le eletise eletise.
• Immunofixation: o se metotia e mafai ai ona suʻesuʻeina ma faʻapipiʻiina monoclonal immunoglobulins
• Immunoglobulin su'ega: o se faiga e vavae'ese ai polotini mai le plasma ma fa'ailoa i latou i luga o le fa'avae o tali fa'ama'i e mafai ona iloa latou te maua.

Ona alu lea o le suʻesuʻega e suʻe le mafuaʻaga o le GM. O fa'aaliga eseese ile falema'i, olaola po'o fa'aeletonika e tatau ona fa'ailoa mai ai le tele ole myeloma:

• Pa'u mamafa, tiga o le ponaivi inflammatory, gau pathological
• Anemia, hypercalcemia, faaletonu fatu

O isi faʻaaliga e vave faʻaalia i le hemoopathy:

• Lymphadenopathy, splenomegaly
• Fa'aletonu ile faitau toto: anemia, thrombocytopenia, lymphocytosis tele
• Syndrome d'hyperviscosité

O le GMSI o loʻo faʻamatalaina o le GM e aunoa ma se faʻamaʻi poʻo se fale suʻesuʻe faʻailoga o le hematologic malignancy. I totonu o le falemaʻi masani, o se faʻamaoniga lea o le faʻateʻaina. O taʻiala e faʻaaogaina e faʻamalamalamaina ai se GMSI o:

• Monoclonal vaega fua faatatau <30 g / l 
• Tulaga mautu ile taimi ole vaega monoclonal 
• Ole maualuga ole toto ole isi immunoglobulins
• Leai se fa'aleagaina ponaivi fa'aleagaina, anemia ma fa'aletonu fatuga'o

Ole a'afiaga ole GMSI e fa'atupula'ia ile matua mai le 1% ile 25 tausaga ile sili atu ile 5% ile 70 tausaga.

O le GMSI e masani lava e leai ni fa'aaliga. Ae ui i lea, o le monoclonal antibody e mafai ona fusifusia i neura ma mafua ai le faʻalavelave, tingling, ma vaivaiga. O tagata e maua i le ma'i e sili atu le fa'aleagaina o aano o ponaivi ma gau.

Pe a fesoʻotaʻi le GM ma se isi faʻamaʻi, o faʻamaoniga o faʻamaʻi ia.

E le gata i lea, o monoclonal immunoglobulins e mafai ona mafua ai ni faʻalavelave seasea:

• Amyloidosis: teuina o vaega o polotini monoclonal i totoga eseese ( fatugaʻo, fatu, neura, ate) e mafai ona mafua ai le toilalo o nei totoga.
• Plasma hyperviscosity syndrome: e nafa ma fa'aletonu o le va'ai, fa'ailoga o le neura (tiga ulu, niniva, moe, fa'aletonu le mataala) ma fa'ailoga toto.
• Cryoglobulinemia: faʻamaʻi e mafua ona o le i ai i totonu o le toto o immunoglobulins e faʻafefe pe a oʻo i lalo ole 37 ° le vevela. E mafai ona mafua ai faʻaaliga o le paʻu (purpura, Raynaud's phenomenon, extremity necrosis), polyarthralgia, neuritis ma glomerular nephropathies.

Mo IMGs, e leai se togafitiga e fautuaina. O suʻesuʻega talu ai nei o loʻo faʻaalia ai o IMGT ma le gau o ponaivi e mafai ona manuia mai togafitiga faʻatasi ma bisphosphonates. E ta'i 6 i le 12 masina uma, e tatau i tagata gasegase ona faia se su'esu'ega fa'afoma'i ma fa'atino le su'a ma le urine protein electrophoresis e iloilo ai le fa'agasologa o fa'ama'i.

I isi tulaga, o togafitiga o le mafuaʻaga lea.

I se vaega e oʻo atu i le 25% o mataupu, o le faʻaleleia o se GMSI agai i se faʻamaʻi hematologic mataʻutia o loʻo matauina. O tagata e maua i le GMSI e mulimulita'i i su'ega fa'aletino, toto ma o nisi taimi ole mimi pe a fa'alua ile tausaga e siaki ai pe ono fa'asolo ile ma'i kanesa. Afai e vave iloa le alualu i luma, o auga ma faʻalavelave e mafai ona puipuia pe togafitia muamua.