Nukae nye AL Amyloidosis? Mina míaƒo nu tso dɔléle sia si me afɔku le ŋu!

Èse dɔléle sia si woyɔna be `AL Amyloidosis` ŋu nya kpɔa? Ðewohĩ menye nenemae o, alo? Le nyateƒe me la, esia mebɔ vie o, si fia be menye dɔléle si dzea amesiame dzi o. Gake ne edzɔ la, ate ŋu anye nu sesẽ vie. Kpuie ko la, enye tɔtrɔ le lãmenugbagbevi ƒomevi aɖe si woyɔna be ``Plasma cells`` si le míaƒe ŋutilã me, eye protein siwo wowɔna dometɔ aɖewo dona le wo ɖokui si eye wogena ɖe míaƒe ŋutilã ƒe ŋutinu vovovowo me, si gblẽa nu le wo ŋu. Ele abe mɔ̃ aɖe si nɔ dɔ wɔm nyuie eye kasia wòdze akpa siwo mesɔ o wɔwɔ gɔme ene. Mina míaƒo nu tso esia ŋu tsitotsito vie , elabena ele vevie ŋutɔ be míanya esia.

Nuka tututue nye `AL Amyloidosis`?

`AL amyloidosis` sia nye dɔlélewo ƒe hatsotso gã aɖe si woyɔna be `Amyloidosis` ƒomevi ɖeka. `Amyloidosis` nye dɔléle si mebɔ o si dzɔna ne tɔtrɔ, alo `mutation`, va `plasma cells` me le míaƒe ƒumeŋuɖui me. Plasme lãmenugbagbevi siawo siwo trɔ la wɔa protein ƒomevi aɖe si mesɔ o. Protein siawo, abe ka ƒe bɔl siwo xaxa ene, ƒoa ƒu ɖekae, eye wogena ɖe míaƒe ŋutinu kple lãkusi veviwo me.

Le AL amyloidosis me la, protein ƒomevi si tɔtɔ aleae nye protein si le kɔsɔkɔsɔ si me kɔ. Protein siawo siwo le kɔsɔkɔsɔ bɔbɔe me nye dɔlékuiwutike siwo míaƒe ŋutilã zãna tsɔ wɔa avu kple dɔlélewo ƒe akpa aɖewo. Plasma me lãmenugbagbevi mawo ke siwo ŋu meƒo nu tsoe va yi ye wɔa dɔlékuiwutike siawo.

Zi geɖe la, AL amyloidosis gblẽa nu le míaƒe dzi kple/alo ayiku ŋu wu . Gake ate ŋu agblẽ nu le dɔgboa, dɔgbo, lãmekawo, kple ŋutigbalẽ ŋu ɣeaɖewoɣi.

Nu nyuitɔ kekeakee nye be ne wokpɔ dɔléle sia dze sii kaba eye wodae kaba , woate ŋu akpɔ egbɔ ɣeaɖewoɣi abe dɔléle si nɔa anyi didi ene. Gake ne womewɔ atike nɛ o la, ate ŋu ahe nɔnɔme sesẽ siwo ate ŋu ade ame ƒe agbe afɔku me kple ku gɔ̃ hã vɛ. Eyata esia menye nusi woabu nu tsɛe o.

Nukae ŋɔŋlɔdzesi eve siwo nye `AL` le ŋkɔ sia me fia?

Amyloidosis ƒomevi vovovowo li. Wotsɔ protein si mesɔ o si hea dɔlélea vɛ la ƒe ŋkɔ na ƒomevi ɖesiaɖe. Le AL amyloidosis me la, ŋɔŋlɔdzesi A tsi tre ɖi na amyloidosis eye ŋɔŋlɔdzesi L tsi tre ɖi na protein si woyɔna be light chain si hea dɔlélea vɛ. Amyloidosis ƒomevi bubu siwo wonya nyuie enye AA amyloidosis (si serum amyloid A protein hena vɛ) kple ATTR amyloidosis (si transthyretin hena vɛ).

Aleke `AL amyloidosis` kpɔa ŋusẽ ɖe nye ŋutilã dzii?

Kpuie ko la, AL amyloidosis nye dɔléle si le ʋumenugbagbevi dzĩwo me. Plasma cells nye míaƒe dɔlélenutsiŋutete ƒe akpa aɖe. Woƒe dɔ vevitɔe nye be woawɔ dɔlékuiwutikewo atsɔ awɔ avu kple dɔlékuiwo. Tsɔe be le míaƒe ŋutilã me la, ʋumenugbagbevi dzĩ akpe geɖe siwo wɔa dɔlékuiwutike ƒomevi vovovowo tsɔ wɔa avu kple dɔléle vovovowo. Plasme lãmenugbagbevi ɖeka ma eye wòwɔa lãmenugbagbevi bubu geɖewo.

Le dɔléle siwo me ‘plasma cells’ le me la, ‘plasma cells’ siawo ƒe ƒuƒoƒo aɖe ma le mɔ si dzi womate ŋu aɖu o nu, eye wowɔa ‘antibodies’ ƒomevi ɖeka ƒe agbɔsɔsɔ gã aɖe.

Le AL amyloidosis me la, ne wowɔ dɔlékuiwutikewo la, wowɔa protein kɔsɔkɔsɔ eve siwo woyɔna be kɔsɔkɔsɔ kpekpewo kple kɔsɔkɔsɔ bɔbɔewo. Nusi dzɔna le afisiae nye be plasma lãmenugbagbeviwo wɔa protein siwo me kɔsɔkɔsɔ si me kɔ la sɔ gbɔ akpa. Kɔsɔkɔsɔ bɔbɔe siawo meƒoa wo nɔewo nyuie o eye woƒoa ƒu ɖekae. Woyɔa protein siawo siwo ƒo ƒu ɖekae be amyloid fibrils. Fibril siawo gena ɖe míaƒe ŋutinuwo me. Ɣemaɣie nu gblẽ le ŋutinu mawo ŋu vevie , ɣeaɖewoɣi ale gbegbe be woate ŋu ade woƒe agbe afɔku me.

Amekae ate ŋu axɔ dɔléle sia wu?

Amyloidosis nye dɔléle si mebɔ kura o. Wobu akɔnta be edzea ame 9 va ɖo 14 le miliɔn ɖeka me le United States eye wòdzea ame 5 va ɖo 12 le miliɔn ɖeka me le xexeame ƒe akpa bubuwo. AL amyloidosis bɔ ɖe ŋutsuwo me vie wu nyɔnuwo. Zi geɖe la, edzea amesiwo xɔ wu ƒe 60 hã dzi.Ƒe si woxɔna hafi wokpɔa dɔlélea le mama dedie nu anɔ abe ƒe 64 ene.

Nukae nye `AL amyloidosis` ƒe dzesiwo?

`AL amyloidosis` ateŋu adze menye míaƒe ŋutilã ƒe ŋutinuwo ɖeɖeko dzi o, tso ta vaseɖe afɔwo dzi, ke boŋ abɔ kple afɔwo hã. Zi geɖe la, dɔléle sia ƒe dzesiwo ate ŋu asɔ kple dɔléle bubu siwo mesẽ boo o ƒe dzesiwo. Azɔ hã, dzesiawo dzena blewu ŋutɔ . Eyata ɖewohĩ màde dzesi tɔtrɔ aɖe le wò ŋutilã me enumake o.

Dzesi siwo do ƒome kple ta kple kɔ:

• Ðe nèsena le ɖokuiwò me be taɖuame alo tagbɔ le ƒoƒom (`tagbɔsesẽ`) ne èfɔ?
• Ðe ʋuʋudedi si ƒe amadede nye aŋutiɖiɖi le wò ŋkuwo ŋu alo le wò ŋkume?
• Ðe wò aɖe sena le eɖokui me be yelolo wu alesi wòle dzɔdzɔmeea ?

Dzesi siwo do ƒome kple asiwo kple afɔwo:

Nɔnɔme siawo ate ŋu anɔ asiwo ŋu:

• Ðe nèsea ʋuʋudedi, dzobibi, alo ʋuʋudedi le wò asiwo mea? Esiawo ateŋu anye nɔnɔme aɖe si woyɔna be ``Peripheral Neuropathy`` ƒe dzesiwo.
• Ðe wò asibidɛwo le ʋuʋum eye wole ʋuʋum ? Esiawo ateŋu anye ``Carpal Tunnel Syndrome`` ƒe dzesiwo.

Dzesi siwo dzena le afɔwo kple afɔwo ŋu ate ŋu anye:

• Ðe wò afɔwo alo afɔwo vuvua ?
• Ðe wò afɔwo sena le wo ɖokui me be agbe mele wo ŋu oa ?

Tsɔ kpe ɖe eŋu la, àte ŋu ade dzesii be yexɔa abi bɔbɔe , ʋu dona bɔbɔe, alo ŋutigbalẽa ƒe amadede trɔna aŋutiɖiɖi le afisiwo ʋuʋudedi le.

Dzesi siwo fia be dzi kple lãkusi me kuxiwo le ŋuwò:

Dzesi siwo le ete ate ŋu anye dɔléle bubuwo ƒe dzesiwo hã, gake kpɔ nyuie:

• Dzi ƒe tsotso: Seselelãme abe ɖe dzia le ƒoƒom hele du dzi ene.
• Gbɔgbɔ kpui (gbɔgbɔtsixetsixe): Akɔta ƒe ʋuʋu, abe ɖe màte ŋu agbɔ ya sesĩe o ene.
• Akɔtave : Vevesese le akɔta ƒe akpa sia akpa. Vevesese sia ate ŋu asẽ, aɖiɖi, alo ava yi eye wòadzo. Akɔtave ate ŋu anye dzidɔ ƒe dzesi hã, eyata ne akɔtave le ŋuwò si xɔa wu miniti atɔ̃ eye medzona ne ègbɔ ɖe eme alo nèzã atike o la, ƒo ka na 911 enumake alo nàna ame aɖe nakplɔ wò ayi kɔdzi.
• Ðeɖiteameŋu : Ðeɖiteameŋu ƒe seselelãme ale gbegbe be màte ŋu awɔ wò gbesiagbedɔwo gɔ̃ hã o, abe ɖe ŋusẽ aɖeke megale wò ŋutilã me o ene.

Dzesi siwo fia be dɔgbo alo dɔgbo me kuxiwo le ŋuwò:

AL amyloidosis ate ŋu akpɔ ŋusẽ ɖe wò nuɖuɖu hã dzi. Dzesiawo ate ŋu anye:

• Nuɖuɖumadzroame : Amesiame ƒe nuɖuɖudzroame trɔna tso ɣeyiɣi yi ɣeyiɣi. Gake ne nuɖuɖu megadzroa wò o ŋkeke geɖe susu aɖeke manɔmee la, ate ŋu anye dɔgbo me kuxi aɖe si do ƒome kple AL amyloidosis ƒe dzesi.
• Ʋuʋudedi alo gas si gbɔ eme : Ʋuʋudedi kple gas nye dzesi siwo bɔ, gake ɣeaɖewoɣi la, wodoa ŋukpe ame. Gake ne gas si nɔa anyi ɖaa le ŋuwò la, ate ŋu anye dɔgbo me kuxi aɖe si do ƒome kple AL amyloidosis ƒe dzesi.
• Dɔmeɖui : Aɖuɖɔtoe nye aɖuɖɔtoe si mede zi etɔ̃ o kwasiɖa sia kwasiɖa. Ne dɔmeɖui le ŋuwò wu kwasiɖa etɔ̃ la, enye kuxi si ŋu wòle be nàƒo nu tsoe na wò ɖɔkta.
• Aɖuɖɔtoe : Aɖuɖɔ si me tsi le. Ne aɖuɖɔtoetetedɔ gakpɔtɔ li la, ele be nàgblɔe na wò ɖɔkta hã.

Dzesi siwo fia be ayiku alo tsinyegoe me kuxiwo le ŋuwò:

Àte ŋu ade dzesi tɔtrɔ le wò aɖuɖɔ ƒe dzedzeme ŋu alo zi nenie wòhiã be nàɖe aɖuɖɔ. Dzesi siawo ate ŋu anye:

• Ðe nèkpɔa kuɖɔ geɖe le wò aɖuɖɔ me wu alesi nèkpɔnɛ ɖaa?
• Ðe mèle aɖuɖɔ tom fũu oa , alo ɖe wòle be nàfɔ le zã me hafi aɖu aɖuɖɔa ?

Nukae hea AL amyloidosis vɛ?

Míeƒo nu tso esia ŋu vie va yi. `AL amyloidosis` tso `plasma cells` siwo wɔa `antibodies`, protein siwo woyɔna be `heavy chains` kple `light chains` gbɔ, ne wowɔ `light chain` protein mawo wògbɔ eme. `Kɔsɔkɔsɔ siwo me kɔ` siawo ƒoa ƒu nyuie o, ƒoa ƒu ɖekae, eye wowɔa `amyloid fibrils` siwo emegbe wogena ɖe míaƒe ŋutinuwo me. Ɣemaɣie kuxiawo dzea egɔme.

Aleke ɖɔktawo wɔa dea dzesi AL amyloidosis?

Ðɔktawo zãa dodokpɔ geɖe tsɔ dea dzesi AL amyloidosis. Dodokpɔ vevitɔe nye be woaɖe lãkusi alo ŋutinu sue aɖe si wosusu be dɔlélea gblẽ nu le eŋu. Woyɔa esia be biopsy. Woate ŋu ade lãmenugbagbeviwo ƒe dɔwɔwɔ ɖe hatsotsowo me ale:

• Bone marrow biopsy : Esia bia be woaɖe ƒumeŋuɖui si le ƒu me ƒe kpɔɖeŋu sue aɖe.
• Ayiku me lãmenugbagbeviwo ɖeɖe : Woate ŋu axɔ ayiku ƒe lãkusi sue ʋɛ aɖewo.
• Dzi ƒe lãmenugbagbeviwo ɖeɖe : Woate ŋu axɔ dzi ƒe lãmeka sue ʋɛ aɖewo.
• Fat pad biopsy : Woɖea ami sue aɖe tso ƒodoa me. Esia nye dodokpɔ si le bɔbɔe vie.

Dodokpɔ bubuwo

Wò ɖɔkta ate ŋu awɔ dodokpɔ bubuwo atsɔ akpɔ alesi wò ŋutinuwo le dɔ wɔm nyuie. Esiawo ate ŋu anye:

• Ʋudodokpɔ : Wowɔa esiawo tsɔ léa ŋku ɖe ale si wò ayiku, dzi, kple aklã le dɔ wɔm ŋu, eye be woatsɔ akpɔ kɔsɔkɔsɔ fafɛ agbɔsɔsɔme si le wò ʋu me.
• Aɖuɖɔtoe dodokpɔ : Zi geɖe la, esia nyea aɖuɖɔtoe dodokpɔ si wowɔna gaƒoƒo 24 sɔŋ. ȃoa wò aɖuɖɔ nu ƒu le aƒeme eye nètsɔa eƒe aɖuɖɔa vaa wò ɖɔkta gbɔ. Dodokpɔ sia léa ŋku ɖe wò ayikuwo ŋu be amyloidosis gblẽ nu le wo ŋu hã.
• Electrocardiogram (ECG): ECG dzidzea dzia ƒe elektrikŋusẽ ƒe dɔwɔwɔ.
• Echocardiogram : Woyɔa esia ɣeaɖewoɣi be dzi ƒe ultrasound, eye wozãa gbeɖiɖi ƒe ƒutsotsoe siwo ƒe tsɔtsɔme sesẽ tsɔ dzidzea dzia ƒe ʋuʋu.
• Dzitodzito me MRI (Cardiac Magnetic Resonance Imaging) : Dodokpɔ sia ate ŋu aɖe dzia ƒe foto siwo me kɔ ŋutɔ, siwo me kɔ nyuie.

Aleke wodaa AL amyloidosis?

Ðɔktawo daa AL amyloidosis be wòaɖe wò dzesiwo dzi akpɔtɔ, akpɔ ŋutinuwo ƒe gbegblẽ dzi, eye wòana amyloid protein siwo mesɔ o ƒe ƒoƒo ƒu blewu alo atɔ te wo .

Ðɔktawo ate ŋu atsɔ atike, dɔlélenutsiŋutete, alo steroid ada AL amyloidosis. Wonaa atike ɖeka alo eve ame akpa gãtɔ, eye zi geɖe la, wotsɔa steroid kpena ɖe eŋu. Atike siawo wɔa dɔ ɖekae tsɔ tsrɔ̃a ʋumenugbagbevi dzĩ siwo wɔa protein siwo le kɔsɔkɔsɔ si me kɔ la me.

Vevietɔ la, togbɔ be atike siawo ate ŋu atɔ te AL amyloidosis ƒe ŋgɔyiyi alo ana wòanɔ blewu hã la, womate ŋu aɖe amyloid fibrils siwo ƒo ƒu ɖe wò ŋutinuwo me xoxo la ɖa o. Ne wonya dze atikewɔwɔ gɔme ko la, wò ŋutɔ wò dɔlélenutsiŋutete ate ŋu aɖe protein siawo siwo mesɔ o ɖa vivivi. Numekulawo le dɔlékuiwutike yeye siwo nye monoclonal antibodies siwo ate ŋu aɖe ka siawo ɖa hã dim.

Wò ɖɔkta aƒo nu na wò hã tso nenye be ``ƒumeŋuɖui dodo na ame`` alo ``lãmenugbagbeviwo dodo na ame ateŋu aɖe vi na wò.`` Togbɔ be esiawo nye atikewɔwɔ siwo sesẽ wu hã la, ateŋu akpɔ dzidzedze ŋutɔ na ame aɖewo.

Ðe woate ŋu axe mɔ ɖe `AL amyloidosis` ƒe dzɔdzɔ nua?

Nublanuitɔe la, ao. Mɔ aɖeke meli si dzi woato axe mɔ ɖe AL amyloidosis alo amyloidosis ƒomevi bubu aɖeke nu o. AL amyloidosis dzɔna ne ʋumenugbagbevi dzĩ ɖeka va zu nusi mesɔ o eye wòdze mama gɔme le mɔ si dzi womate ŋu aɖu o nu. Esia menye nusi dzi míate ŋu aɖu o.

Ɣeyiɣi didi kae nàte ŋu anɔ agbe kple `AL amyloidosis`?

Fifia ɖɔktawo le kpekpem ɖe amesiwo ŋu AL amyloidosis le ŋu be woanɔ agbe didi wu. Le ame aɖewo gome la, AL amyloidosis nye dɔléle si nɔa anyi didi, alo esi nɔa anyi didi, si woate ŋu atsɔ atike akpɔ egbɔe. Gake ele vevie be míaɖo ŋku edzi be esia nye dɔléle sesẽ aɖe ŋutɔ eye ate ŋu ahe atikewɔwɔ me nɔnɔme siwo ate ŋu ade ame ƒe agbe afɔku me vɛ.

Esi wònye be AL amyloidosis nye dɔléle si mebɔ o ta la, esesẽna na ɖɔktawo be woanya ɣeyiɣi didi si tututu amesi ŋu dɔlélea le ate ŋu anɔ agbe. Ne AL amyloidosis le ŋuwò la, bia wò ɖɔkta tso wò ŋgɔdonya ŋu le atikewɔwɔ vɔ megbe. Wò ɖɔkta ate ŋu ana nyatakaka nyuitɔ kekeake wò tso nusi nàkpɔ mɔ na ŋu.

Aleke malé be na ɖokuinye?

Ne AL amyloidosis le ŋuwò la, wò ɖɔkta ƒe susu anɔ wò dzesiwo dada kple dɔlélea nu tsotso alo eƒe ŋgɔyiyi blewu ŋu koŋ. Ðewohĩ ahiã be nàyi edzi anɔ atikewo nom, eye atikewɔwɔ mawo ate ŋu agblẽ nu le ame ŋu. Mɔ ɖeka si dzi nàto akpɔ ɖokuiwò dzie nye be nàsrɔ̃ nu tso wò atikewɔwɔwo ƒe nugbegblẽ le ame ŋu kple alesi nàwɔ akpɔ wo gbɔe ŋu. Aɖaŋuɖoɖo bubu aɖewoe nye esi:

• Dɔdamɔnu aɖewo ate ŋu agblẽ nu le wò nuɖuɖudzroame ŋu.Ne nenemae la, bia wò ɖɔkta tso nuɖuɖu nyui siwo akpe ɖe ŋuwò be ŋusẽ nanɔ ŋuwò le atikewɔwɔ me ŋu. Nuɖuɖu si da sɔ ɖuɖu le vevie ŋutɔ.
• Dze agbagba nàgbɔ ɖe eme geɖe . Gbɔɖeme le vevie na ŋutilãa ne ele avu wɔm kple dɔléle sia tɔgbe.
• Kamedede nye mɔ nyui aɖe si dzi nàto ana wò susu nanɔ dzidzɔ kpɔm eye wòakpe ɖe ŋuwò nàlé wò kpekpeme si sɔ. Gake bia wò ɖɔkta be wòaɖo aɖaŋu na wò le kamedede siwo matsɔ ɖeke le eme na wò ŋutilã fũ o ŋu.
• Esi wònye be AL amyloidosis nye dɔléle si mebɔ o ta la, àte ŋu ase le ɖokuiwò me be yetsi akogo eye yeɖe ye ɖokui ɖe aga . Ðewohĩ ame bubuwo mase dɔlélea gɔme o. Bia wò ɖɔkta tso kpekpeɖeŋunaha alo ɖoɖo siwo wowɔna na amesiwo ŋu AL amyloidosis le ŋu. Nuƒoƒo na ame mawo tɔgbi ate ŋu anye ŋusẽtsoƒe gã aɖe.

Mlɔeba la, nya vevi ʋɛ aɖewo (Take-Home Message) .

AL amyloidosis (Amyloid light chain alo primary amyloidosis) nye dɔléle si mebɔ o si tsoa light chain protein siwo mesɔ o ƒe tsɔtsɔ ɖe míaƒe ŋutilã ƒe ŋutinuwo kple lãmenugbagbeviwo me gbɔ. Ate ŋu anye dɔléle sesẽ ŋutɔ , si wokpɔa egbɔ ɣeaɖewoɣi abe dɔléle si nɔa anyi didi ene, gake ate ŋu ahe atikewɔwɔ me nɔnɔme siwo ate ŋu ade ame ƒe agbe afɔku me hã vɛ.

Gake mègatsi dzi o, ɖɔktawo le mɔnu yeyewo dim be yewoakpe ɖe amesiwo ŋu AL amyloidosis le ŋu be woanɔ agbe didi wu eye woanɔ agbe nyuie wu. Ne AL amyloidosis le ŋuwò la, bia wò ɖɔkta tso dɔdamɔnu dodokpɔ yeyewo kple numekuku siwo woawɔ atsɔ ada dɔlélea ŋu. De dzesii kaba kple edada nye nu vevitɔ. Ele vevie hã be nànɔ susu nyui me eye nàwɔ ɖe wò ɖɔkta ƒe aɖaŋuɖoɖowo dzi.

` AL amyloidosis, amyloidosis gbãtɔ, plasma lãmenugbagbeviwo, kɔsɔkɔsɔ me protein siwo me kɔ, amyloid fibrils, ayikudɔ, dzidɔ