Monoclonal gammopathy (GM) inotsanangurwa nekuvapo muserum uye / kana weti ye monoclonal immunoglobulin. Inogona kusanganiswa nehemopathy yakaipa, kana zvisina kudaro inonzi monoclonal gammopathy yekukosha kusingazivikanwi (GMSI).

Nekuongororwa, kuongororwa kwemicrobiological kunoita kuti zvikwanise kuona iyo monoclonal immunoglobulin mukuwanda. Clinical, biological and radiological manifestations inogona kunongedzera kune hemopathy nepo kuongororwa kweGMSI kuri kusiyanisa kuongororwa.

tsananguro

Monoclonal gammopathy (GM) inotsanangurwa nekuvapo muserum uye / kana weti ye monoclonal immunoglobulin. Immunoglobulins mapuroteni ari muplasma yevanhu ane immune properties. Iwo anogadzirwa mumasero eplasma, masero eiyo lymphoid system inoumbwa muspleen uye lymph nodes. GM saka inopupurira kupararira kwe clone yemasero eplasma anobudisa monoclonal immunoglobulin.

Types

GMs inogona kuiswa muzvikamu zviviri:

• Monoclonal gammopathies inobatanidza nehematologic malignancies
• Monoclonal gammopathies yezvisina kukosha kukosha (GMSI)

Zvinokonzera

Kune monoclonal gammopathies yakabatana neinouraya hemopathies, zvikonzero zvikuru ndezvi:

• Multiple myeloma: bundu remapfupa emapfupa akaumbwa kubva mukuwanda kwemasero eplasma asina kujairika
• Macroglobulinemia (chirwere cheWaldenström): kuvapo muhuwandu husina kujairika muplasma ye macroglobulin
• B lymphoma

GMSI inogona kusanganiswa neakasiyana-siyana asiri akashata pathologies:

• Autoimmune zvirwere (rheumatoid polyathritis, Sjögren's syndrome, systemic lupus)
• Utachiona hwehutachiona (mononucleosis, chickenpox, HIV, hepatitis C)
• Utachiona hwehutachiona (endocarditis, osteomyelitis, tuberculosis)
• Zvirwere zveparasitic (leishmaniasis, marariya, toxoplasmosis)
• Zvirwere zvisingaperi senge chisingaperi cholecystitis (kuzvimba gallbladder)
• Zvimwe zvakasiyana-siyana zvakadai semhuri hypercholesterolemia, chirwere cheGaucher, Kaposi's sarcoma, lichen, chirwere chechiropa, myasthenia gravis (kusagadzikana kwekutapurirwa kwetsinga kubva mutsinga kuenda kumhasuru), anemia kana thyrotoxicosis.

chirwere

GM inowanzoonekwa sezvinei, panguva yekuongororwa kwerabhoritari kune zvimwe zvikonzero.

Kuti uone iyo yakawandisa monoclonal agent, iyo inonyanya kubatsira miedzo ndeye:

• Electrophoresis yemapuroteni eSerum: nzira inobvumira kuziva uye kuparadzanisa mapuroteni eserum pasi pekuita kwemunda wemagetsi.
• Immunofixation: nzira inobvumira kuonekwa uye kutaipa kwemonoclonal immunoglobulins
• Immunoglobulin assay: maitiro anoparadzanisa mapuroteni kubva kuplasma uye anoazivisa pahwaro hwekuona maitiro eimmunological aanogadzira.

Zvadaro kuongororwa kunoenda kuburikidza nekutsvaga chikonzero cheGM. Makiriniki akasiyana-siyana, biological kana radiological kuratidzwa kunofanirwa kuratidza akawanda myeloma:

• Kurasikirwa uremu, kuzvimba mapfupa marwadzo, pathological fractures
• Anemia, hypercalcemia, renal kukundikana

Zvimwe zviratidzo zvinongonongedza kune hemopathy:

• Lymphadenopathy, splenomegaly
• Zvisizvo muhuwandu hweropa: anemia, thrombocytopenia, yakawandisa lymphocytosis
• Syndrome d'hyperviscosité

GMSI inotsanangurwa seGM pasina chero zviratidzo zvekiriniki kana laboratory zvehematologic malignancy. Muchirongwa chekiriniki, uku ndiko kuongororwa kwekusabatanidzwa. Maitiro anoshandiswa kutsanangura GMSI ndeaya:

• Monoclonal chikamu chiyero <30 g / l 
• Kugadzikana kwehukama nekufamba kwenguva kwechikamu chemonoclonal 
• Yakajairika serum level yemamwe immunoglobulins
• Kusavapo kwekukuvara kwemapfupa anoparadza, anemia uye chirwere cheitsvo

Chiitiko cheGMSI chinowedzera nezera kubva pa1% pamakore makumi maviri nemashanu kusvika kupfuura 25% kupfuura makore makumi manomwe.

GMSI inowanzoita asymptomatic. Nekudaro, iyo monoclonal antibody inogona kusunga kune tsinga uye kukonzera chiveve, kurira, uye kushaya simba. Vanhu vane chirwere ichi vanowanzova nekuparadzwa kwemapfupa emapfupa uye kuputsika.

Apo GM inobatanidzwa nechimwe chirwere, zviratidzo ndezviya zvechirwere.

Uyezve, monoclonal immunoglobulins inogona kukonzera matambudziko asingawanzo:

• Amyloidosis: madhipoziti ezvimedu zvemonoclonal mapuroteni munhengo dzakasiyana (itsvo, moyo, tsinga, chiropa) izvo zvinogona kuva chikonzero chekutadza kwenhengo idzi.
• Plasma hyperviscosity syndrome: inokonzera kukanganisa kwekuona, zviratidzo zvetsinga (musoro, dzungu, kurara, kusvinura) uye zviratidzo zvekubuda ropa.
• Cryoglobulinemia: zvirwere zvinokonzerwa nekuvapo muropa re immunoglobulins iyo inoputika kana tembiricha iri pasi pe37 °. Vanogona kukonzera kuratidzwa kweganda (purpura, Raynaud's phenomenon, extremity necrosis), polyarthralgia, neuritis uye glomerular nephropathies.

Kune maIMG, hapana kurapwa kunokurudzirwa. Zvidzidzo zvenguva pfupi yapfuura zvinoratidza kuti IMGTs ine hukama kurasikirwa kwepfupa inogona kubatsirwa nekurapa nemabisphosphonates. Mwedzi mitanhatu kusvika ku6 yega yega, varwere vanofanira kuongororwa nekliniki uye kuita serum uye urini protein electrophoresis kuti vaongorore kufambira mberi kwechirwere.

Mune zvimwe zviitiko, kurapwa ndiko kwechikonzero.

Muchikamu chinosvika makumi maviri neshanu muzana chezviitiko, kushanduka kweGMSI kune chirwere chinotyisa chehematologic chinoonekwa. Vanhu vane GMSI vanoteverwa nekuongororwa kwemuviri, ropa uye dzimwe nguva weti kanenge kaviri pagore kuti vatarise kufambira mberi kwechirwere chegomarara. Kana kufambira mberi kukaonekwa nekukurumidza, zviratidzo uye matambudziko anogona kudzivirirwa kana kurapwa pachine nguva.