Iiprotheni ze-Bence-Jones zizinto ezincinci ze-antibodies ezinokuthi zifunyenwe ngokusebenzisa uvavanyo lomchamo, ikakhulukazi ukuxilonga nokujonga izigulane ezine-myeloma ezininzi, uhlobo lomhlaza wegazi.
Multiel Myeloma
I-myeloma emininzi ngumhlaza owenza uhlobo lweseli elimhlophe elibizwa nge-plasma cell. Iiseli zePlasma yiiseli ezenza iziza-mzimba zikuncede ukulwa nezifo.
Kwinqanaba le-plasma ekhiqiza umntu oqhelekileyo, umntu unokuthi ukuhlukahluka kwezoshishino zabo. Benza zonke iintlobo ezahlukeneyo zeeprotheni ze-antibody.
I-myeloma yamanye yenye yezifo ezininzi ezinokuvelisa "ikhefu elilodwa lamaseli e-plasma." Cinga ngelinye iqela njengento efana nesihlwele samaseli amawele afanayo. Esi sihlwele asifani. I-clone enye yamaseli ngokubanzi yenza iiprotheni ezifanayo.
M-proteins
Xa kuxoxwa ngeeprotheni ze-Bence-Jones, amanye amagama amaninzi aqhelekileyo asetyenziswa, afana namaprotheni e-myeloma, i-M-proteins, i-paraproteins, i-free-cell immunoglobulin kunye ne-M-spike. La magama onke afana nawo, kodwa awanakuguqulwa.
I-'M 'kwiprotheni ye-M imele i-monoclonal, oku kuthetha ukuba ukuqokelela kweeprotheni zivela kwiqela elilodwa leeseli ezivelisa abantu. Ezi iiseli zonke zivelisa iinjenge-antibodies ezifanayo okanye iinxalenye zengqungquthela.
Iintambo Zokukhanya
Ama-antibodies yiloo nto izazinzulu zibiza ngokuba ngama-immunoglobulins - ukuthetha ngomzimba kukunceda balwe nezifo, kunye ne-globulin, ithetha ukuba yiiprotheni.
Ezi zi-immunoglobulins zihlala zikhulu kakhulu, kodwa ziqulethwe ngamacandelo amancinci ahlanganiswayo. Ama-antibodies ngokwenene aqulethwe ngamancinci amancinci amancinci, abizwa ngokuba ngamaketanga anzima kunye neetambo zokukhanya.
Kulo mfanekiso ukhankanywe ngentla apha, ungabona umntu opheleleyo olwaziyo okanye i-immunoglobulin ngaphambili, kodwa umntu ngamnye olwaziyo unamalungu ambalwa.
Ingxenye ebomvu yiyo "iinqununu ezinzima" kunye neengxenye zegolide "ziiminyango ezilula." Kulo mfanekiso, iinqwelo zegolide eziqhelekileyo ziyakwazi ukuba yiiprotheni ze-Bence-Jones kumntu onamagama amaninzi okanye enye imeko.
Iiprotheni zeBence-Jones ziyinxalenye ekhethekileyo yeqoqo elincinci le-antibody (i-chain chains) . Ubukho beetyhewu ezikhanyayo ngobuninzi obunomchamo abuqhelekanga. Ngokuqhelekileyo, umlinganiselo wemizuzu emfutshane yeprotheni yokukhanya ingatholakala kwi-urine. Amaketanga okukhanya kumchamo angakhula ngezizathu ezininzi, nangona kunjalo, kwaye akubona bonke aba ngumhlaza.
Amaketanga okuKhanya eMonoclonal
Ngoku, kwimeko yeproteins yeBence-Jones, kubalulekile ukukhumbula ukuba ezi zikhonkwane ezilula azikho nje uhlobo oludala lweetambo zokukhanya- zizololu hlobo oluqhelekileyo.
Iiprotheni zeBence-Jones ke ngoko zi ...
- I-Monoclonal (ivela kwiqela elilodwa leeseli)
- Amaketanga okukhanya omonoclonal (iikopi ezininzi ze-anti-part-part-part part)
- Efumaneka kumchamo kwaye idibene neemeko ezahlukeneyo, kuquka i-myeloma eninzi.
Iimeko
Iiprotheni ze-Bence-Jones zihlobo ezithile ze-M-proteins, kwaye zincinci ngokwaneleyo ukuba zidlule kwi-system filtration system, izintso. Oku kuthetha ukuba, xa ezi zikhonkwane ezikhanyayo zivela ngobuninzi, ziya kudlula kwigazi lakho ukuya kumchamo wakho.
Kukho imeko ezininzi apho i-protein yeBence Jones ifumaneka kumchamo womntu, kuquka oku kulandelayo:
- I-myeloma yamaninzi - I- myeloma yamaninzi ngumdlavuza wegazi apho kukho i-cell-overproduction yeeseli ze-plasma-iiseli ezivela kwi-B lymphocytes kwimathambo amathambo. Iiseli zePlasma yiiseli ezenza iintsholongwane ezisikhusela kwiintsholongwane ezithile kunye neebhaktheriya. Phakathi kwama-50 no-80% abantu abane-myeloma baya kuba nokuvavanya umchamo okwenzela izi proteins.
- I-macroglobulinemia yeWaldenström - I -macroglobulinemia yeWaldenstrom ngumdla ongaqhelekanga ovela kwiiseli ze-B (uhlobo lweseli legazi elimhlophe). Iiseli ze-B zihamba kwinkqubo yokuba iiseli ze-plasma ezivelisa abantu. Esi sifo, umhlaza uvela kwi-cell ye-B, phambi kwegama apho liba yiseli ye-plasma; ngoko, i-macroglobulinemia yaseWaldenström iyahluke kwi-myeloma yam, kodwa ezi zimbini, ezinye iimeko ezifanayo, kwaye zombini zingabangela iiprotheni zeBence-Jones.
- I-MGUS-MGUS imele i-gammopathy ye-monoclonal engabalulekanga. Esi sifo sinokuba ngumlandeleli we-myeloma kwaye iquka ukunyuka kwenani lamaseli e-plasma, kodwa akukho nenye yezinye iimpawu ezinxulumene ne-myeloma.
Ukubaluleka
Iiprotheni ze-Bence-Jones zikhona malunga nezigulane ezimbini kwizigulane ezintathu ezine-myeloma ezininzi. I-myeloma yamaninzi ngumhlaza wegazi obandakanya ukuveliswa kwemveliso yee-plasma. Njengokuba umhlaza wamaphaphu wawuza kuba neminxeba engavamile yomhlaza wamaphaphu, i-myeloma iquka iindili ze-plasma ezingaphezulu.
Iiseli zePlasma yileli iiseli ezenza iintsholongwane zisincede silwe nezifo. Noko ke, iiseli zeplasma ezinomhlaza, kodwa, azenzi ii-antibodies ezikunceda ukulwa nezifo, kwaye endaweni yoko, iiseli ze-plasma ezingaphezu kwee-antibodies ezifanayo.
Ukugqithisa kwama-immunoglobulin emzimbeni kunokuba yinkinga evela kwinani leendidi ezahlukeneyo, esinye sezifo zesifo . Ngokuqhelekileyo, amanqindi omzimba aphezulu kakhulu ukuba ahlanjwe kwiibhulo ezintso. Iiprotheni zeBence-Jones zincinci ngokwaneleyo ukungena kwiiyunithi zokucoca ezintso. Kwizintso, ezi proteins zakha kwaye zenze iingxaki zeengtso. Ezinye iintlobo zeeprotheni ziyakwazi ukulimaza iinjongo ngale ndlela kunabanye. Kukho neminye imiphumo yezicwangciso zokukhanya ezigqithiseleyo ezinokuchaphazela amalungu ahlukeneyo omzimba.
Ukuba uye wafumanisa ukuba unesifo esiphumela kwiimveliso zamaprotheni alula, uogqirha wakho uya kubeka iliso malunga neengxaki ezinxulumene nazo. Enye yezinto ezinokuthi zenzeke ngamaketanga okukhanya ngokugqithisileyo kuthiwa yi-amyloid-chain chain-amyloidosis, apho izikhonkwane zokukhanya zidibanisa kwaye zenze iifomethi zezinto ezibizwa ngokuthi i-amyloid, ezingabangela umonakalo kwizitho ezahlukeneyo.
Uvavanyo lweeUni
Uvavanyo lweeprotheni zeBence-Jones ziquka ukuqokelela umchamo wakho iiyure ezingama-24 ukujonga ubungqina beeprotheni. Iiprotheni zeBence-Jones zithiwa nguHenry Bence Jones, onguchwepheshe wezilwanyana oqala ukufumana iimpawu ezingavamile zezi sampuli zamanzi emkhatsini we-1800s. Kukho iintlobo ezimbini zeetyunithi zokukhanya ezibizwa ngokuba yi-kappa kunye ne-lambda. Uluhlobo oluthile lwenketho lokukhanya lungabaluleka.
ILizwi
Ukuba usanda kufunyanwa, sele ufumane ixesha lokufunda ngesifo sakho kwaye ubuze imibuzo. Fumana uxhaso lwabahlobo bakho nabathandekayo. Uze uxhomeke kwithemba. Ukunyanga kwama- khansa yegazi kuye kwaphuculwa ngokuphawulekayo kwiminyaka yakutshanje, kwaye unyango olutsha ngoku luvavanyo lwezonyango.
> Imithombo:
> Basile U, Gulli F, Torti E, et al. Ukuphononongwa kwendlela yokuhlola inkqubo ye-Bence Jones iphente analysis. KwiKliniki yeMil Lab Med. 2016; 54 (11): e331-e333.
> Cook Cook, Macdonald DHC. Ulawulo lweparaproteinemia. I-Postgraduate Medical Journal . 2007; 83 (978): 217-223.
> Kyle, R., Larson, D., Therneau, T. et al. Inkqubo yekliniki yokukhanyisa i-myeloma ezininzi (idiopathic Bence Jones proteinuria): ukufundwa kweqela eliphindaphindiweyo. Lancet Hematology . 2014. 1 (1): e28-36.