Ingaba le protein ye-M ikwayingxaki nakwizintso zakho? (I-Monoclonal Gammopathy of Renal Significance - MGRS) Masithethe ngale nto!

Sonke siyazi ukuba izintso zethu sisitho esibaluleke kakhulu esenza umsebenzi waso buthule. Nangona kunjalo, ngamanye amaxesha ezi zintso zinokugula buthule. Namhlanje siza kuthetha ngesifo esibangela isifo sezintso, esinokubonakala sinzima kancinci xa usiva igama, kodwa kulula ukuliqonda. Oku kubizwa ngokuba yiMGRS (Monoclonal Gammopathy of Renal Significance) . Nangona igama lide kancinci, ungakhathazeki, masiliqonde ngokulula.

Yintoni iMGRS ngokulula?

Kulungile, makhe sijonge ukuba yintoni i-MGRS. Ngamafutshane, i-MGRS liqela leemeko ezonakalisa izintso. Lo monakalo uvela kuba ezinye zeeseli zeplasma zomzimba wethu, okanye iiseli ze-B (ezi ziintlobo zeeseli ezimhlophe zegazi), zenza iikopi ezininzi zazo, okanye ii-clones . Cinga ngayo njengomatshini wokufotokopa owenza iikopi ezininzi zephepha elifanayo.

Iiseli zeplasma eziveliswa ngale ndlela zivelisa uhlobo lwe -antibody olubizwa ngokuba yi -M protein . Ezi protein ze-M zigcinwa kwiindawo ezahlukeneyo zomzimba wethu, ingakumbi izintso, kwaye ziqale ukuzonakalisa.

Into ebalulekileyo kukuba i-MGRS ayingomhlaza. Nangona kunjalo, inani elincinci labantu abane-MGRS linokuba nomhlaza ekugqibeleni, njenge- multiple myeloma . Ngoko ke kubalulekile ukukwazi oku.

Ngaba kukho iintlobo zeMGRS?

Ewe, kukho iintlobo ezingaphezu kweshumi elinesibini zeemeko ze-MGRS. Ezi zahlulwe ngokwenxalenye yentso echaphazeleka yiproteni ye-M. Kukho iindidi ezintathu eziphambili:

1. Izifo zeGlomerular

Uhlobo oluqhelekileyo lwe-MGRS yi -glomerulopathies . Ezi zonakalisa imithambo yegazi emincinci ngaphakathi kwezintso. Zifana neengxobo ezikwisihluzo samanzi. Ezinye zezifo eziwela kolu didi ziquka:

• I-amyloidosis yezintso. Oku kuquka iintlobo ezibizwa ngokuba yi-AL amyloidosis, i-AH amyloidosis, kunye ne-AHL amyloidosis.
• I-cryoglobulinemic glomerulonephritis (CG).
• I-Fibrillary glomerulopathy (FGN).
• I-immunotactoid glomerulopathy (ITG).
• Izifo ze-glomerular ezinxulumene neeproteni ze-monoclonal. Oku kuquka iimeko ezibizwa ngokuba yi-'light chain' kunye ne-'heavy chain deposition diseases'.

2. Izifo zeTubulointerstitial

Ezi zifo zichaphazela iityhubhu (iityhubhu) ezithwala izondlo kunye nolwelo oluhluzwa zizintso lubuyela egazini, kunye nezicubu ezizingqongileyo. Eminye imizekelo ibandakanya:

• I-Light chain Fanconi syndrome (LCFS).
• I-Light chain proximal tubulopathy (LCPT) engenazo iikristale.
• I-histiocytosis egcina iikristale.

3. Iimeko ezichaphazela imithambo yegazi ngaphakathi kwezintso (Intra-renal vascular lesions)

Ezi meko zichaphazela imithambo yegazi kwizintso. Umzekelo yi- atypical hemolytic uremic syndrome (aHUS) . Oku kwenzeka xa imithambo yegazi emincinci kwizintso ivalwa ngamahlwili egazi.

Ixhaphake kangakanani iMGRS?

I-MGRS yimeko engaqhelekanga kakhulu . Kuqikelelwa ukuba ichaphazela ngaphantsi kwe-1% yabemi. Ixhaphake kakhulu kubantu abangaphezu kweminyaka engama-60 ubudala .

Zithini iimpawu ze-MGRS?

Iimpawu ze-MGRS zinokwahluka ngokuxhomekeke kuhlobo lwe-MGRS onayo. Ezinye zeempawu eziqhelekileyo ziquka:

• Ndiziva ndidiniwe kakhulu (ndidiniwe) .
• Ukudumba emilenzeni esezantsi, emaqatheni, okanye ezinyaweni. Ngamanye amaxesha isisu, izandla, okanye ubuso bunokudumba.
• I-Nephrotic syndrome iyenzeka. Oku kubonakala ngokukhupha iiproteni emchameni kunye namanqanaba aphezulu e-cholesterol egazini.
• Umchamo onegwebu okanye onesepha . Oku kungaba luphawu lweproteni kumchamo (proteinuria).
• Ukuchama rhoqo kukufuna ukuchama rhoqo.
• Igazi kumchamo (i-hematuria) okanye umchamo opinki.
• Isicaphucaphu nokuhlanza.
• I-POEMS syndrome . Le yimeko engaqhelekanga.
• Ukuba ndindisholo, ukurhawuzelela, okanye ubuthathaka kwimilenze (ezi ziimpawu ze-neuropathy).
• Ukutshintsha kombala wolusu (amabala akhanyayo okanye amnyama).
• Ubunzima bokuphefumla (ukuphelelwa ngamandla).

Yintoni ebangela i-MGRS?

Eyona nto ibangela i-MGRS kukonakala kwezintso okubangelwa ziiproteni ze-M ezikhankanyiweyo apha ngasentla. Iiproteni ze-M luhlobo lwe-antibody engasebenzi kakuhle, kwaye iveliswa ngendlela engafanelekanga. Zenziwe ngee-clones ezonakeleyo (iikopi) zeeseli ze-plasma, uhlobo lweeseli ezimhlophe zegazi, okanye iiseli ze-B.

Abanye abantu banokuba nezi clones zeeseli zeplasma ezenza iiproteni ze-M ngaphandle kokulimaza amalungu omzimba. Le meko ibizwa ngokuba yiMonoclonal Gammopathy of Undetermined Significance (MGUS) . Nangona kunjalo, kubantu abane-MGRS, ezi proteni ze-M ziyaqokelelana ezintsoni zize zonakalise.

Yintoni eyona nto ibangela i-MGRS?

Iingcali azikayazi kakuhle into ebangela i-MGRS. Iingxaki zokuzikhusela komzimba , utshintsho lwemfuza , usulelo, kunye nezinto ezingqongileyo zonke zicingelwa ukuba zinegalelo kuyo.

Ziziphi iingxaki ezinokwenzeka ngenxa ye-MGRS?

Ezi plasma cell clones kunye neMGRS zinokubangela umonakalo kwizintso, nto leyo enokubangela iingxaki ezinkulu. Ezinye zezi ziquka:

• Isifo sezintso esingapheliyo (CKD) .
• Isifo sezintso (sezintso) esikwinqanaba lokugqibela .
• Ukungasebenzi kakuhle kwezintso .
• Ukukhula kube zezinye izifo. Kwabanye abantu, inani lee-clones zeeseli zeplasma liyanda kakhulu kangangokuba linokuphuhla libe luhlobo lomhlaza weeseli ezimhlophe zegazi olubizwa ngokuba yi-multiple myeloma (MM) . I-MGRS ingaphuhla ibe zezinye iintlobo zomhlaza, ezifana neWaldenström macroglobulinemia , i-chronic lymphocytic leukemia (CLL) , okanye i-B-cell lymphoma .

Ifunyaniswa njani i-MGRS?

Ugqirha unokuqinisekisa i-MGRS ngokwenza i-biopsy yezintso . Oku kuquka ukuthatha iqhekeza elincinci lesicubu esisezintsoni zakho aze alivavanye ukuze abone ukuba kukho iproteni ye-M. Banokuvavanya neesampuli zegazi lakho, umchamo, okanye umongo wamathambo.

Ngaba kukho unyango lwe-MGRS?

Ewe, kukho unyango lwe-MGRS. Oogqirha bayayinyanga ngamayeza afana ne -chemotherapy yomhlaza. La mayeza abulala iiseli ezinengxaki okanye awathintele ekwenzeni ezinye iikopi. Amanye amayeza anokunceda ekunciphiseni ukudumba. Injongo ephambili yonyango kukugcina izintso zakho zisebenza kwaye zithintele ukuba zonakale.

Kwezinye iimeko ze-MGRS, oogqirha banokwenza utyando lweeseli ze-stem . Oku kuthatha indawo yeeseli zakho ezonakeleyo ngeeseli eziphilileyo. Ukuba izintso zakho zonakele kakhulu, ungadinga i-dialysis okanye utyando lwezintso .

Amayeza athile kunye nonyango olusetyenziswa kwi-MGRS

Uhlobo lwamayeza owanikwayo luya kuxhomekeka kuhlobo lwe-MGRS onayo. Nazi ezinye iindlela zonyango:

• Ii-antibodies ze-monoclonal - umz. i-rituximab, i-daratumumab.
• Izithinteli zeProteasome - umz. iBortezomib, iCarfilzomib, iXazomib.
• Amayeza okulawula amajoni omzimba - umz. i-thalidomide, i-lenalidomide, i-pomalidomide.
• Ii-alkylating agents - umz. i-cyclophosphamide, i-bendamustine, i-melphalan.
• Ii-corticosteroids - umz. i-prednisone, i-dexamethasone.
• Ukufakelwa kweeseli ze-Autologous stem.

Zithini iziphumo ebezingalindelekanga zonyango?

Unyango olufana ne-chemotherapy lunokubangela iziphumo ebezingalindelekanga ezininzi ezingathandekiyo, ngamanye amaxesha kwanomonakalo wamalungu omzimba. Yiyo loo nto oogqirha bekhetha unyango ngononophelo ukuze bafumane inzuzo enkulu kulo kwaye banciphise umngcipheko weziphumo ebezingalindelekanga ezinzulu. Ezinye zeziphumo ebezingalindelekanga onokuzifumana ziquka:

• I-anemia ( ukungabikho kwegazi).
• Ukopha.
• Ukuqhinwa.
• Urhudo.
• Ndidiniwe.
• Umngcipheko okhulayo wosulelo lwentsholongwane, iintsholongwane kunye nefungus.
• Ukutya akunancasa.
• Isicaphucaphu nokuhlanza.

Ngaba i-MGRS ingathintelwa?

Ngenxa yokuba singazi kakuhle ukuba yintoni ebangela i-MGRS, okwangoku akukho ndlela ithile yokuyithintela.

Yintoni onokuyilindela ukuba une-MGRS?

Nangona i-MGRS yimeko enokunyangwa, ayipheli ngokupheleleyo. Ingaphinda ivele emva konyango. Ukuba izintso zakho zonakele kakhulu, unokufuna ukufakelwa izintso.

Malunga ne-18% yabantu abane-MGRS kamva baya kuba nomhlaza obizwa ngokuba yi-multiple myeloma. Ke ngoko, ugqirha wakho uya kukujonga rhoqo ngeesampuli zegazi nomchamo, njengoko oku kunokubonwa kwangethuba ukuba kwenzeka.

Ungaphila ixesha elingakanani neMGRS?

Ixesha onokuphila ngalo ne-MGRS lixhomekeke kuhlobo lwe-MGRS onayo, ubukhali bayo, kunye nendlela osabela ngayo kunyango. Abantu abanomonakalo omncinci kwizintso banembono engcono kunabantu abanomonakalo omkhulu. Umzekelo, kolunye uphando, abantu abane-amyloidosis enxulumene ne-MGRS baphila umyinge weminyaka eli-10 emva kokufunyaniswa ukuba bane-amyloidosis. Kodwa abantu abangenayo i-amyloidosis baphila ixesha elide kakhulu. Kwakhona, abantu abasabela kakuhle kunyango baneziphumo ezilungileyo.

Ndingazinyamekela njani?

Qiniseka ukuba ulandela naziphi na iingcebiso ugqirha wakho akunika zona zokunyamekela impilo yezintso zakho ekhaya. Oku kungabandakanya ukunciphisa izinto ezifana notywala, kunye nokulawula izinto ezinokubangela isifo sezintso, ezifana noxinzelelo lwegazi oluphezulu kunye nesifo seswekile. Nangona ezi zinto zingenakunyanga ngqo i-MGRS, zinokunceda ukunciphisa umngcipheko wokonakala kwezintso ngakumbi. Ugqirha wakho unokukunceda nokulawula iziphumo ebezingalindelekanga zonyango.

Ndifanele ndimbone nini ugqirha?

Ukuba uneempawu zengxaki yezintso, ezinje ngokudumba, umchamo onegwebu okanye opinki, ukuchama rhoqo, okanye ukuchama kancinci kakhulu, qiniseka ukuba ubona ugqirha.

Ndingayibuza yiphi imibuzo ugqirha wam?

Kunganceda ukubuza ugqirha wakho le mibuzo:

• Ziziphi iindlela zonyango ezikhoyo zohlobo lwe-MGRS endinazo?
• Ndingenza ntoni ukuthintela ukonakala kwezintso?
• Zeziphi iimpawu ezintsha okanye eziya ziba mandundu ekufuneka ndiziqaphele?

Okokugqibela, eyona nto ibalulekileyo (Umyalezo Wokuya Ekhaya)

I-MGRS yimeko enzima kakhulu enokonakalisa izintso ngendlela enokuba yingozi ebomini. Kodwa khumbula, ayinguye wonke umntu one-MGRS oza kuba nomhlaza. Ugqirha wakho uza kukujonga rhoqo aze athathe amanyathelo okuchonga nokunyanga naziphi na iingxaki ezintsha ngokukhawuleza . Unyango olutsha ngoku lwenze kwaba nokwenzeka ukuphucula umgangatho wobomi bakho kunye nokunciphisa umngcipheko wezifo ezinzulu. Ngoko ke ungoyiki, landela icebiso likagqirha wakho, kwaye ujongane nale meko.

` MGRS, iMonoclonal Gammopathy yokubaluleka kwezintso, isifo sezintso, iiproteni ze-M, iiseli zeplasma, i-Kidney Biopsy, i-Nephrotic Syndrome, i-Multiple Myeloma