Monoclonal gammopathy (GM) e hlalosoa ke ho ba teng ka har'a serum le / kapa moroto oa monoclonal immunoglobulin. E ka amahanngoa le malignant hemopathy, ho seng joalo e bitsoa monoclonal gammopathy ea bohlokoa bo sa tsejoeng (GMSI).

Bakeng sa tlhahlobo, litlhahlobo tsa likokoana-hloko li etsa hore ho khonehe ho khetholla monoclonal immunoglobulin ka bongata bo feteletseng. Liponahatso tsa bongaka, tsa baeloji le tsa radiological li ka supa ho hemopathy athe tlhahlobo ea GMSI ke tlhahlobo e fapaneng.

Tlhaloso

Monoclonal gammopathy (GM) e hlalosoa ke boteng ba serum le / kapa moroto oa monoclonal immunoglobulin. Li-immunoglobulins ke liprotheine tse ka har'a plasma ea motho tse nang le thepa ea 'mele ea ho itšireletsa mafung. Li entsoe ka lisele tsa plasma, lisele tsa lymphoid system tse entsoeng ka spleen le lymph nodes. Ka hona GM e fana ka bopaki ba ho ata ha clone ea lisele tsa plasma tse hlahisang monoclonal immunoglobulin.

Types

Li-GM li ka aroloa ka mekhahlelo e 'meli:

• Li-monoclonal gammopathies tse amanang le malignancies ea mali
• Monoclonal gammopathies of undetermined significance (GMSI)

Lisosa

Bakeng sa li-gammopathies tsa monoclonal tse amanang le malignant hemopathies, lisosa tse kholo ke:

• Multiple myeloma: hlahala ea moko oa masapo e entsoeng ka ho ata ha lisele tse sa tloaelehang tsa plasma
• Macroglobulinemia (lefu la Waldenström): ho ba teng ka bongata bo sa tloaelehang ho plasma ea macroglobulin
• B lymphoma

GMSI e ka amahanngoa le mafu a fapaneng a seng kotsi:

• Maloetse a Autoimmune (rheumatoid polyathritis, Sjögren's syndrome, systemic lupus)
• tšoaetso ea kokoana-hloko (mononucleosis, khohopox, HIV, hepatitis C)
• Tšoaetso ea baktheria (endocarditis, osteomyelitis, lefuba)
• Tšoaetso ea likokoana-hloko (leishmaniasis, malaria, toxoplasmosis)
• Maloetse a sa foleng joalo ka cholecystitis e sa foleng (ho ruruha ha gallbladder)
• Maemo a mang a fapaneng a kang hypercholesterolemia ea lelapa, lefu la Gaucher, sarcoma ea Kaposi, lichen, lefu la sebete, myasthenia gravis (bothata ba phetiso ea methapo ea kutlo ho tloha methapong ho ea mesifa), phokolo ea mali kapa thyrotoxicosis.

Diagnostic

Hangata GM e fumanoa ka tšohanyetso, nakong ea liteko tsa laboratori tse etsoang ka mabaka a mang.

Ho khetholla monoclonal agent e ngata haholo, liteko tse molemo ka ho fetisisa ke tsena:

• Electrophoresis ea liprotheine tsa serum: mokhoa o lumellang ho khetholla le ho arola liprotheine tsa serum tlasa ts'ebetso ea lebala la motlakase.
• Immunofixation: mokhoa o lumellang ho fumanoa le ho thaepa ha monoclonal immunoglobulins
• Immunoglobulin assay: ts'ebetso e arolang liprotheine ho plasma le ho li tsebahatsa motheong oa karabelo ea immunoglobulin eo ba e hlahisang.

Ebe tlhahlobo e tsoela pele ho batla sesosa sa GM. Lipontšo tse fapaneng tsa bongaka, tsa baeloji kapa tsa radiological li lokela ho fana ka maikutlo a multiple myeloma:

• Ho lahleheloa ke boima ba 'mele, bohloko ba masapo a ho ruruha, fractures ea pathological
• Anemia, hypercalcemia, ho hloleha ha renal

Lipontšo tse ling hang-hang li supa hemopathy:

• Lymphadenopathy, splenomegaly
• Lintho tse sa tloaelehang palo ea mali: phokolo ea mali, thrombocytopenia, lymphocytosis e feteletseng
• Syndrome d'hyperviscosité

GMSI e hlalosoa e le GM ntle le matšoao leha e le afe a kliniki kapa a laboratori a lefu la malignancy. Ka tloaelo ea kliniki, sena ke tlhahlobo ea ho qheleloa ka thoko. Mekhoa e sebelisoang ho hlalosa GMSI ke:

• Sekhahla sa motsoako oa monoclonal <30 g / l 
• Botsitso bo amanang le nako ea karolo ea monoclonal 
• Boemo bo tloaelehileng ba serum ea li-immunoglobulins tse ling
• Ho ba sieo ha masapo a senyang a senya, phokolo ea mali le bothata ba liphio

Ketsahalo ea GMSI e eketseha ka lilemo ho tloha ho 1% ho lilemo tse 25 ho feta 5% ho feta lilemo tse 70.

GMSI hangata ha e na matšoao. Leha ho le joalo, antibody ea monoclonal e ka tlama methapong 'me ea baka bohatsu, ho tsikinyeha le ho fokola. Batho ba nang le lefu lena ba na le menyetla e mengata ea ho senyeha ha lisele tsa masapo le ho robeha.

Ha GM e amahanngoa le lefu le leng, matšoao ke a lefu lena.

Ho feta moo, monoclonal immunoglobulins e ka baka mathata a sa tloaelehang haholo:

• Amyloidosis: li-deposit tsa likaroloana tsa liprotheine tsa monoclonal likarolong tse fapaneng (liphio, pelo, methapo, sebete) e ka ba sesosa sa ho hlōleha ha litho tsena.
• Plasma hyperviscosity syndrome: e ikarabella bakeng sa mathata a pono, matšoao a methapo (hlooho, ho tsekela, ho otsela, mathata a ho falimeha) le matšoao a tšollo ea mali.
• Cryoglobulinemia: mafu a bakoang ke boteng ba mali a immunoglobulins a phallang ha mocheso o le ka tlase ho 37 °. Li ka baka ponahalo ea letlalo (purpura, Raynaud's phenomenon, extremity necrosis), polyarthralgia, neuritis le glomerular nephropathies.

Bakeng sa li-IMG, ha ho phekolo e khothalletsoang. Liphuputso tsa morao-rao li bontša hore li-IMGT tse nang le tahlehelo ea masapo e amanang le tsona li ka rua molemo kalafong ea li-bisphosphonates. Likhoeli tse ling le tse ling tse 6 ho isa ho tse 12, bakuli ba lokela ho hlahlojoa le ho etsa electrophoresis ea protheine ea serum le moroto ho lekola tsoelo-pele ea lefu lena.

Maemong a mang, phekolo ke eona sesosa.

Karolong ea ho fihlela ho 25% ea linyeoe, phetoho ea GMSI ho ea ho lefu le kotsi la hematologic e hlokomeloa. Batho ba nang le GMSI ba lateloa ka liteko tsa 'mele, mali le moroto ka linako tse ling hoo e ka bang habeli ka selemo ho hlahloba tsoelo-pele e ka bang teng boemong ba mofetše. Haeba tsoelo-pele e fumanoa kapele, matšoao le mathata a ka thibeloa kapa a phekoloa kapele.