Yintoni i-AL Amyloidosis? Makhe sithethe ngesi sifo singaqhelekanga ngamazwi alula

Ngaba wakha wacinga ukuba ukuziva udiniwe rhoqo, ukuba nemilenze evuvukileyo, kunye nobunzima bokuphefumla kuyinto eqhelekileyo? Ngamanye amaxesha, kunokubakho imeko enzima ngakumbi emva kwezi mpawu esingazivanga kangako ngazo. Namhlanje, sithetha ngesifo esingaqhelekanga, kodwa ekufanele ukuba usiqaphele. Yiyo leyo i-AL Amyloidosis. Eli gama lisenokuvakala lisoyikisa kancinci, kodwa masithethe ngalo ngokulula nangokucacileyo.

Ngamafutshane, yintoni i-AL amyloidosis?

Khawucinge ukuba sineefektri ezincinci emizimbeni yethu, esizibiza ngokuba ziiseli zeplasma . Ezi zikwi-bone mongo wethu. Umsebenzi wazo ophambili kukwenza amajoni abizwa ngokuba zii-antibodies ukulwa neentsholongwane ezingena emizimbeni yethu. Le yinxalenye ebaluleke kakhulu kwinkqubo yethu yokuzikhusela komzimba.

Kodwa kwimeko ebizwa ngokuba yi-AL amyloidosis, kukho isiphene esincinci kwezi seli zeplasma. Ngenxa yesi siphene, ezo seli zivelisa uhlobo lweproteni olungasebenzi kakuhle, oluphukileyo, nolungasebenzanga kakuhle. Oku kubizwa ngokuba yiproteni yetsheyini ekhanyayo .

Ezi protein ze-light chain ezigobileyo ziyahlangana, zenze ii-tangles kunye nezakhiwo ezincinci, ezine-fibrous. Ezi proteins ziyaqokelelana kwizitho ezahlukeneyo emizimbeni yethu, njengentliziyo, izintso, imithambo-luvo kunye namathumbu. Njengothuli oluvala umbhobho, xa ezi proteins ziqokelelana, ezo zitho azikwazi ukusebenza kakuhle. Oku sikubiza ngokuba yi-AL amyloidosis.

Kutheni oku kubizwa ngokuba yi-AL?

Kukho iintlobo ezahlukeneyo ze-amyloidosis. Uhlobo ngalunye lubizwa ngegama leproteni engaqhelekanga eyibangelayo. Unobumba u-A umele i-Amyloidosis kwaye unobumba u- L umele i- Light Chain .

Esi sifo sichaphazela njani umzimba?

Kukho amawaka eentlobo ezahlukeneyo zeeseli zeplasma emizimbeni yethu. Uhlobo ngalunye lwenza uhlobo oluthile lwe-antibody yokulwa nentsholongwane ethile.

Kwi-AL amyloidosis, iseli enye yeplasma ivelisa isiphene, kwaye loo seli iyahlukana ngokungalawulekiyo, ivelise amawaka eeseli ezinesiphene. Ngayinye kwezi seli yenza iproteni yetsheyini yokukhanya enesiphako esithethe ngayo ngaphambili.

Olu buninzi beprotheyini luhamba egazini lethu luze luqale ukugcwala kwizitho zethu zomzimba. Ezi fibre zigciniweyo zibizwa ngokuba yi -amyloid fibrils . Ezi zibangela ukuba izitho zomzimba zibe nkulu kwaye zingasebenzi kakuhle. Ekuhambeni kwexesha, oku kunokubangela umonakalo omkhulu onokuba yingozi nakubomi.

Ngubani onokuthi afumane esi sifo?

Esi sisifo esingaqhelekanga . Ehlabathini lonke, sichaphazela inani elincinci labantu, phakathi kwe-5 kunye ne-12 kwisigidi.

• Okungakumbi kumadoda kunabafaziUkuthambekela kokwakha kukhulu kakhulu.
• Le meko ixhaphake kakhulu kubantu abaneminyaka engaphezu kwama-60 ubudala. Ubudala obuqhelekileyo bokuxilongwa bumalunga neminyaka engama-64 ubudala.

Ziziphi iimpawu ezinokuchongwa?

Ingxaki enye ngesi sifo kukuba iimpawu zaso zihlala zifana nezezinye izifo eziqhelekileyo. Kwaye ezi mpawu zibonakala kancinci kakhulu. Ngoko ke usenokungawuboni umahluko omkhulu ekuqaleni. Makhe sibone ukuba zeziphi ezi mpawu kwitheyibhile engasezantsi.

Ugqirha uluchonga njani ngokuchanekileyo olu sifo?

Emva kokumamela iimpawu zakho, ukuba ugqirha urhanela ukuba unesifo, uza kwenza iimvavanyo ezininzi ukuqinisekisa ukuba unesifo.

Uvavanyo olubaluleke kakhulu koku yi-biopsy . Oku kuquka ukuthatha iqhekeza elincinci kakhulu lesicubu okanye ilungu elithile elicingelwa ukuba lichaphazelekile size silihlolisise phantsi kwe-microscope. Oku kusenza sibone ukuba ikhona na iproteni ye-amyloid. Uvavanyo lwe-biopsy ludla ngokwenziwa ukuze:

• I-Bone marrow biopsy: Kuthathwa isampuli encinci kumongo wethambo ngaphakathi kwethambo.
• I-biopsy yezintso: Kuthathwa iziqwenga ezincinci kakhulu zezicubu kwintso.
• I-biopsy yentliziyo: Kuthathwa iziqwenga ezincinci ezimbalwa zemisipha yentliziyo.
• I-biopsy ye-Abdominal Fat Pad: Kuthathwa iqhekeza elincinci lethishu kulwelo olunamafutha phantsi kolusu lwesisu.

Uvavanyo olongezelelweyo

Ukongeza koku, kwenziwa uvavanyo olongezelelekileyo ukuze kufunyaniswe ukuba monakalo mkhulu kangakanani owenziwe kumalungu akho omzimba.

• Uvavanyo lwegazi: Olu vavanyo lunceda ekuboneni indlela ezisebenza ngayo izintso, intliziyo, nesibindi, kunye nokulinganisa ubungakanani beprotheyini yetsheyini yokukhanya egazini.
• Uvavanyo lomchamo: Isampuli yomchamo, edla ngokuqokelelwa kwixesha leeyure ezingama-24, iyavavanywa ukuze kubonwe ukuba akukho monakalo kwizintso.
• I-ECG (i-Electrocardiogram): Ilinganisa umsebenzi wombane wentliziyo.
• I-Echocardiogram: I-ultrasound scan yentliziyo. Oku kujonga izinto ezifana nokuhamba kwentliziyo kunye nobukhulu bayo.
• I-Cardiac MRI: Olu vavanyo lwenziwa ukuze kufunyanwe umfanekiso ocacileyo noneenkcukacha zentliziyo.

Zithini iindlela zonyango zoku?

Kukho iinjongo eziphambili ezininzi zokunyanga i-AL amyloidosis. Enye kukulawula iimpawu. Enye kukulawula umonakalo wamalungu omzimba. Kwaye okona kubaluleke kakhulu, kukuyeka ukuveliswa kweproteni engaqhelekanga .

Oogqirha badla ngokusebenzisa amayeza anjenge -chemotherapy , i-immunotherapy , okanye ii-steroids ukutshabalalisa iiseli ze-plasma ezineengxaki, ezithi emva koko ziyeke ukuveliswa kweeproteni ezintsha ze-light chain.

Kwezinye iimeko, kuxhomekeke kwimeko yesigulana , kunokucetyiswa ukuba utyando lwe-bone marrow okanye utyando lwe-stem cell . Konke oku kugqitywa ngugqirha okunyangayo.

Ungaphila ixesha elingakanani nale sifo?

Le yingxaki kubantu abaninzi. Enyanisweni, unyango olutsha ngoku luye lwandisa kakhulu ixesha lokuphila kwabantu abane-AL amyloidosis. Kwabanye abantu, iye yaba sisifo esingapheliyo esinokulawulwa ngamayeza.

Kodwa asifanele silibale ukuba esi sisifo esibi kakhulu. Imeko yekamva yesigulana imiselwa zizinto ezininzi, njengobungakanani bomonakalo kumalungu omzimba xa isifo sifunyenwe, kunye nendlela umzimba osabela ngayo kunyango.

Ngaba esi sifo singathintelwa? Ndingazinyamekela njani?

Ngelishwa, i-AL amyloidosis ayisosifo esinokusithintela. Sibangelwa kukuguquka okungacwangciswanga kwiseli yeplasma. Kodwa xa isifo sifunyenwe, kukho izinto ezimbalwa onokuzenza ukuze uzinyamekele.

• Ukutya okunempilo: Ezinye iindlela zonyango zinokubangela ukuphelelwa ngumdla wokutya. Kodwa ukutya okunempilo kubalulekile ukuze umzimba wakho uhlale unamandla. Thetha nogqirha wakho malunga nokutya okufanelekileyo kuwe.
• Phumla ngokwaneleyo: Nika umzimba wakho ixesha lokuphola. Lala ngokwaneleyo kwaye uphumle.
• Ukuzilolonga okufanelekileyo:Ukuzilolonga kulungile engqondweni nasemzimbeni. Kodwa buza ugqirha wakho ukuba zeziphi izilolonga ezikhuselekileyo nezifanelekileyo emzimbeni wakho.
• Inkxaso yengqondo: Xa unesifo esingaqhelekanga esifana nesi, ungaziva unesizungu kwaye unesizungu kuba abanye abaqondi. Yinto eqhelekileyo leyo. Buza ugqirha wakho ngamaqela enkxaso kubantu abanale meko. Ukwabelana ngamava akho nabanye ngamandla amakhulu.

Nangona i-AL amyloidosis sisifo esibi kakhulu, kunokwenzeka ukulawula nokuphila nale meko ngokuyifumanisa kwangethuba, ukufumana unyango olufanelekileyo, nokuzinyamekela kakuhle.