I-Monoclonal gammopathy (GM) ichazwa ngokuba khona ku-serum kanye / noma umchamo we-monoclonal immunoglobulin. Ingahlotshaniswa ne-hemopathy eyingozi, ngaphandle kwalokho ibizwa ngokuthi i-monoclonal gammopathy yokubaluleka okunganqunyiwe (GMSI).

Ukuze uthole ukuxilongwa, ukuhlolwa kwe-microbiological kwenza kube nokwenzeka ukuhlonza i-monoclonal immunoglobulin ngobuningi ngokweqile. Ukubonakaliswa komtholampilo, kwebhayoloji kanye ne-radiological kungakhomba ku-hemopathy kuyilapho ukuxilongwa kwe-GMSI kuwukuxilongwa okuhlukile.

Incazelo

I-Monoclonal gammopathy (GM) ichazwa ngokuba khona ku-serum kanye / noma umchamo we-monoclonal immunoglobulin. Ama-Immunoglobulins angamaprotheni ku-plasma yomuntu anezakhiwo zokuzivikela. Ahlanganiswa kumaseli e-plasma, amangqamuzana ohlelo lwe-lymphoid akhiwe ku-spleen kanye nama-lymph nodes. Ngakho-ke i-GM ifakazela ukwanda kwe-clone yamaseli e-plasma akhiqiza i-monoclonal immunoglobulin.

izinhlobo

Ama-GM angahlukaniswa abe yizigaba ezi-2:

• I-Monoclonal gammopathies ehambisana ne-hematological malignancies
• I-Monoclonal gammopathies of undetermined significance (GMSI)

Izimbangela

Nge-monoclonal gammopathies ehambisana ne-hemopathies eyingozi, izimbangela eziyinhloko ziyi:

• I-Myeloma eminingi: isimila somnkantsha wethambo esakhiwe ngenxa yokwanda kwamaseli e-plasma angajwayelekile
• I-Macroglobulinemia (isifo sika-Waldenström): ukuba khona ngamanani angavamile ku-plasma ye-macroglobulin
• B lymphoma

I-GMSI ingahlotshaniswa nama-pathologies angewona amabi:

• Izifo ze-autoimmune (i-rheumatoid polyathritis, i-Sjögren's syndrome, i-systemic lupus)
• Ukutheleleka ngegciwane (i-mononucleosis, inkukhu, i-HIV, i-hepatitis C)
• Ukutheleleka kwamagciwane (i-endocarditis, i-osteomyelitis, isifo sofuba)
• Izifo ezithathelwanayo (i-leishmaniasis, i-malaria, i-toxoplasmosis)
• Izifo ezingamahlalakhona njenge-cholecystitis engapheli (ukuvuvukala kwe-gallbladder)
• Ezinye izimo ezihlukahlukene ezifana ne-hypercholesterolemia yomndeni, isifo sikaGaucher, i-Kaposi's sarcoma, i-lichen, isifo sesibindi, i-myasthenia gravis (ukuphazamiseka kokudluliswa kwe-nerve impulses isuka ku-nerve iye emisipha), i-anemia noma i-thyrotoxicosis.

kwesifo

I-GM ivame ukutholwa ngengozi, ngesikhathi sokuhlolwa kwaselabhorethri okwenziwa ngezinye izizathu.

Ukukhomba i-ejenti ye-monoclonal ngokweqile, ukuhlolwa okuwusizo kakhulu yizi:

• I-Electrophoresis yamaprotheni e-serum: inqubo evumela ukuhlonza nokuhlukanisa amaprotheni e-serum ngaphansi kwesenzo sensimu kagesi.
• I-Immunofixation: inqubo evumela ukutholwa nokuthayipha kwe-monoclonal immunoglobulins
• I-Immunoglobulin assay: inqubo ehlukanisa amaprotheni ku-plasma futhi iwahlonze ngesisekelo sokusabela okutholakala kwe-immunoglobulin akukhiqizayo.

Khona-ke ukuxilongwa kuhamba ngokubheka imbangela ye-GM. Ukubonakaliswa okuhlukahlukene komtholampilo, kwebhayoloji noma kwe-radiological kufanele kuphakamise i-myeloma eminingi:

• Ukuncipha kwesisindo, ubuhlungu bamathambo okuvuvukala, ukuphuka kwe-pathological
• I-anemia, i-hypercalcemia, ukwehluleka kwezinso

Ezinye izibonakaliso zikhomba ngokushesha ku-hemopathy:

• I-Lymphadenopathy, i-splenomegaly
• Ukuphazamiseka kwenani legazi: i-anemia, i-thrombocytopenia, i-lymphocytosis ngokweqile
• I-syndrome ye-hyperviscosité

I-GMSI ichazwa ngokuthi i-GM ngaphandle kwanoma yiziphi izimpawu zomtholampilo noma zaselabhorethri ze-hematological malignancy. Esimisweni somtholampilo, lokhu ukuxilongwa kokukhishwa. Imibandela esetshenziselwa ukuchaza i-GMSI yilezi:

• Isilinganiso sengxenye ye-Monoclonal <30 g / l 
• Ukuzinza okuhlobene ngokuhamba kwesikhathi kwengxenye ye-monoclonal 
• Izinga le-serum elivamile lamanye ama-immunoglobulins
• Ukungabikho kokulimala kwamathambo okulimazayo, i-anemia kanye nokuphazamiseka kwezinso

Izehlakalo ze-GMSI zikhula ngeminyaka yobudala isuka ku-1% eminyakeni engama-25 iye ngaphezu kuka-5% ngaphezu kweminyaka engama-70.

I-GMSI ngokuvamile ayinazimpawu. Kodwa-ke, i-monoclonal antibody ingabopha emithanjeni futhi ibangele ukuba ndikindiki, ukuluma, kanye nokuba buthakathaka. Abantu abanalesi sifo maningi amathuba okuthi bacekelwe phansi izicubu zethambo kanye nokuphuka.

Uma i-GM ihlotshaniswa nesinye isifo, izimpawu yilezo zesifo.

Ngaphezu kwalokho, i-monoclonal immunoglobulins ingabangela izinkinga ezingavamile:

• I-Amyloidosis: idiphozithi yezingcezu zamaprotheni e-monoclonal ezingxenyeni ezahlukene (izinso, inhliziyo, izinzwa, isibindi) okungaba imbangela yokwehluleka kwalezi zitho.
• I-Plasma hyperviscosity syndrome: inesibopho sokuphazamiseka kokubona, izimpawu zemizwa (ikhanda elibuhlungu, isiyezi, ukozela, ukuphazamiseka kokuqapha) kanye nezimpawu zokopha.
• I-Cryoglobulinemia: izifo ezibangelwa ukuba khona kwegazi kwama-immunoglobulins akhuphuka lapho izinga lokushisa lingaphansi kwama-37 °. Zingabangela ukubonakaliswa kwesikhumba (i-purpura, i-Raynaud's phenomenon, i-extremity necrosis), i-polyarthralgia, i-neuritis kanye ne-glomerular nephropathies.

Kuma-IMG, akukho ukwelashwa okunconywayo. Ucwaningo lwakamuva lubonisa ukuthi ama-IMGT anokulahleka kwethambo okuhlobene angase azuze ekwelashweni ngama-bisphosphonates. Njalo ezinyangeni eziyisi-6 kuya kweziyi-12, iziguli kufanele zihlolwe futhi zenze i-electrophoresis ye-serum ne-urine protein ukuze kuhlolwe ukuqhubeka kwesifo.

Kwezinye izimo, ukwelashwa kuyimbangela.

Engxenyeni efinyelela ku-25% yamacala, ukuvela kwe-GMSI maqondana nesifo se-hematological esiyingozi kuyabonwa. Abantu abane-GMSI balandelwa ukuhlolwa komzimba, igazi futhi ngezinye izikhathi umchamo izikhathi ezimbili ngonyaka ukuze kubhekwe ukuthi kungenzeka yini ukuqhubeka esimweni somdlavuza. Uma ukuqhubekela phambili kutholakala kusenesikhathi, izimpawu nezinkinga zingavinjelwa noma zelashwe kusenesikhathi.