ʻO ka monoclonal gammopathy (GM) i wehewehe ʻia e ka loaʻa ʻana o ka serum a / a i ʻole ka mimi o kahi immunoglobulin monoclonal. Hiki ke hoʻopili ʻia me kahi hemoopathy maʻi, inā ʻaʻole ia i kapa ʻia he monoclonal gammopathy of undetermined significance (GMSI).

No ka hōʻoia ʻana, hiki i nā hoʻokolohua microbiological ke ʻike i ka immunoglobulin monoclonal i ka nui. Hiki i nā hōʻike maʻi, biological a me radiological ke kuhikuhi i kahi hemoopathy aʻo ka hōʻailona o kahi GMSI he hōʻailona ʻokoʻa.

Definition

Ua wehewehe ʻia ka gammopathy monoclonal (GM) e ka loaʻa ʻana o ka serum a me / a i ʻole ka mimi o kahi immunoglobulin monoclonal. ʻO nā immunoglobulins nā protein i loko o ka plasma kanaka i loaʻa nā waiwai pale. Hoʻopili ʻia lākou i loko o nā pūnaewele plasma, nā pūnaewele o ka ʻōnaehana lymphoid i hoʻokumu ʻia i loko o ka spleen a me nā lymph nodes. No laila ke hōʻike nei ʻo GM i ka hoʻonui ʻana o kahi clone o nā cell plasma e hana ana i kahi immunoglobulin monoclonal.

ano

Hiki ke hoʻokaʻawale ʻia nā GM i 2 mau ʻāpana:

• ʻO nā gammopathies monoclonal e pili ana me nā maʻi maʻi hematologic
• Monoclonal gammopathies of undetermined significance (GMSI)

Nā kumu

No nā gammopathies monoclonal e pili ana me nā hemopathies malignant, ʻo nā kumu nui:

• ʻO ka myeloma lehulehu: ʻo ke koko o ka iwi iwi i hoʻokumu ʻia mai ka hoʻonui ʻia ʻana o nā pūnaewele plasma ʻanoʻole
• ʻO Macroglobulinemia (maʻi o Waldenström): ka loaʻa ʻana o nā mea ʻino i ka plasma o macroglobulin
• B lymphoma

Hiki ke hoʻopili ʻia ʻo GMSI me nā ʻano pathologies non-malignant:

• ʻO nā maʻi autoimmune (rheumatoid polyathritis, Sjögren's syndrome, systemic lupus)
• ʻO nā maʻi viral (mononucleosis, chickenpox, HIV, hepatitis C)
• ʻO nā maʻi bacteria (endocarditis, osteomyelitis, tuberculosis)
• ʻO nā maʻi parasitic (leishmaniasis, malaria, toxoplasmosis)
• ʻO nā maʻi maʻi e like me ka cholecystitis mau loa (ka ʻeha o ka gallbladder)
• ʻO nā ʻano maʻi ʻē aʻe e like me ka hypercholesterolemia ʻohana, maʻi Gaucher, sarcoma o Kaposi, lichen, maʻi ate, myasthenia gravis (ka maʻi o ka hoʻoili ʻana o nā aʻalolo mai ke aʻalolo i ka ʻiʻo), anemia a i ʻole thyrotoxicosis.

PalekanaEND_LINK

ʻIke pinepine ʻia ʻo GM, i ka wā o nā hoʻokolohua laboratory i hana ʻia no nā kumu ʻē aʻe.

No ka ʻike ʻana i ka mea monoclonal overabundant, ʻo nā hoʻokolohua pono loa:

• Electrophoresis o nā protein serum: kahi ʻenehana e ʻae ai e ʻike a hoʻokaʻawale i nā protein o kahi serum ma lalo o ka hana o kahi kahua uila.
• Immunofixation: kahi ʻenehana e ʻae ai i ka ʻike a me ke kākau ʻana i nā immunoglobulins monoclonal
• Immunoglobulin assay: kahi kaʻina hana e hoʻokaʻawale i nā protein mai ka plasma a ʻike iā lākou ma ke kumu o nā hopena immunological hiki ke ʻike ʻia.

A laila hele ka ʻike ma ka ʻimi ʻana i ke kumu o GM. Pono e hōʻike ʻia nā hōʻike like ʻole o ka maʻi lapaʻau, olaola a radiological paha:

• ʻO ke kaumaha o ke kaumaha, ka ʻeha iwi ʻeha, nā haʻihaʻi pathological
• Anemia, hypercalcemia, hōʻino ʻole o ka renal

Hōʻike koke nā hōʻike ʻē aʻe i kahi hemoopathy:

• Lymphadenopathy, splenomegaly
• ʻO nā mea kūponoʻole i ka helu koko: anemia, thrombocytopenia, lymphocytosis nui
• ʻO Syndrome d'hyperviscosité

Ua wehewehe ʻia ʻo GMSI ma ke ʻano he GM me ka ʻole o nā hōʻailona lapaʻau a i ʻole nā ​​​​hōʻailona o ka hematologic malignancy. Ma ka maʻamau maʻamau, he hōʻailona kēia o ka wehe ʻana. ʻO nā pae hoʻohālike i hoʻohana ʻia e wehewehe i kahi GMSI:

• Monoclonal helu helu <30 g / l 
• Paʻa pili i ka manawa o ka mea monoclonal 
• Ka pae serum maʻamau o nā immunoglobulins ʻē aʻe
• Ka ʻole o ka pōʻino iwi luku, ka anemia a me ka maʻi puʻupaʻa

Hoʻonui ka nui o ka GMSI me ka makahiki mai 1% ma 25 mau makahiki a ʻoi aku ma mua o 5% ma mua o 70 mau makahiki.

He asymptomatic ka GMSI. Eia nō naʻe, hiki i ka monoclonal antibody ke hoʻopaʻa i nā aʻalolo a hoʻoulu i ka helu ʻana, ke kani ʻana, a me ka nāwaliwali. ʻO ka poʻe me kēia maʻi, ʻoi aku ka nui o ka luku ʻana i ka iwi iwi a me nā haʻihaʻi.

Ke hoʻopili ʻia ʻo GM me kekahi maʻi ʻē aʻe, ʻo ia nā hōʻailona o ka maʻi.

Eia kekahi, hiki i nā immunoglobulins monoclonal ke hana i nā pilikia liʻiliʻi:

• Amyloidosis: ka waiho ʻana o nā ʻāpana o nā protein monoclonal i loko o nā ʻāpana like ʻole (nā puʻuwai, naʻau, nā aʻalolo, ate) hiki ke kumu o ka pau ʻole o kēia mau kino.
• ʻO ka Plasma hyperviscosity syndrome: ʻo ia ke kuleana no nā maʻi ʻike, nā hōʻailona neurological (ʻeha poʻo, dizziness, hiamoe, maʻi makaʻala) a me nā hōʻailona hemorrhagic
• Cryoglobulinemia: nā maʻi i hoʻokumu ʻia e ke komo ʻana i loko o ke koko o nā immunoglobulins e kahe ana i ka wā ma lalo o 37 °. Hiki iā lākou ke hōʻike i ka ʻili (purpura, Raynaud's phenomenon, extremity necrosis), polyarthralgia, neuritis a me glomerular nephropathies.

No nā IMG, ʻaʻole ʻōlelo ʻia ka lāʻau lapaʻau. Hōʻike nā haʻawina hou e hiki ke pōmaikaʻi nā IMGT me ka nalowale o ka iwi mai ka mālama ʻana me nā bisphosphonates. ʻO kēlā me kēia 6 a 12 mau mahina, pono nā mea maʻi e hana i kahi hoʻokolohua lapaʻau a hana i ka serum a me ka urine protein electrophoresis e nānā i ka holomua o ka maʻi.

I nā hihia ʻē aʻe, ʻo ka mālama ʻana ke kumu.

Ma kahi hapa o ka 25% o nā hihia, ʻike ʻia ka ulu ʻana o kahi GMSI i kahi maʻi hematologic malignant. ʻO ka poʻe me ka GMSI e hahai ʻia me ka hoʻāʻo kino, koko a i kekahi manawa ka mimi e pili ana i ʻelua manawa o ka makahiki e nānā i ka piʻi ʻana i kahi maʻi kanesa. Inā ʻike mua ʻia ka piʻi ʻana, hiki ke pale a mālama ʻia nā hōʻailona a me nā pilikia ma mua.