Ðe M protein sia nye kuxi na wò ayikuwo hã? (Monoclonal Gammopathy of Renal Significance - MGRS) Mina míaƒo nu tso esia ŋu!

Mí katã míenya be míaƒe ayikuwo nye ŋutinu vevi aɖe ŋutɔ si wɔa eƒe dɔ le ɖoɖoezizi me. Ke hã, ɣeaɖewoɣi la, ayiku siawo hã ate ŋu adze dɔ le ɖoɖoezizi me. Egbea míele nu ƒom tso dɔléle aɖe si hea ayikudɔ vɛ, si ate ŋu adze nu sesẽ vie ne èse ŋkɔa, gake egɔmesese le bɔbɔe. Woyɔa esia be MGRS (Monoclonal Gammopathy of Renal Significance) . Togbɔ be ŋkɔa didi vie hã la, mègatsi dzi o, mina míase egɔme bɔbɔe.

Nukae nye MGRS ko?

Enyo, mina míalé ŋku ɖe nusi MGRS nye ŋu. Kpuie ko la, MGRS nye nɔnɔme siwo gblẽa nu le ayiku ŋu ƒe ƒuƒoƒo. Nusi gblẽa nu sia dzɔna elabena míaƒe ŋutilã ƒe ʋumenugbagbevi dzĩwo , alo B lãmenugbagbevi aɖewo (esiawo nye ʋumenugbagbevi ɣi ƒomevi aɖewo), wɔa wo ɖokui, alo woƒe nɔnɔmetatawo ƒe kɔpi geɖe akpa. Bu eŋu abe fotokɔpimɔ̃ si le pepa ɖeka ƒe kɔpi geɖe wɔm ene.

Plasma lãmenugbagbevi siwo dzɔna to mɔ sia dzi wɔa dɔlékuiwutike ƒomevi aɖe si woyɔna be M protein . M protein siawo nɔa míaƒe ŋutilã ƒe ŋutinu vovovowo me, vevietɔ ayikuwo, eye wodzea wo dome gbegblẽ gɔme.

Nu vevitɔe nye be MGRS menye kansa o. Gake ame ʋɛ aɖewo siwo ŋu MGRS le ate ŋu axɔ kansa, abe multiple myeloma ene, mlɔeba. Eyata ele vevie be nànya esia.

Ðe MGRS ƒomevi aɖewo lia?

Ẽ, MGRS ƒe nɔnɔme ƒomevi siwo wu wuieve ye li. Woda esiawo ɖe hatsotso siwo me ayiku ƒe akpa si M protein la kpɔa ŋusẽ ɖo nu. Wole hatsotso vevi etɔ̃ me:

1. Glomerular dɔlélewo

MGRS ƒomevi si bɔ wue nye glomerulopathies . Esiawo gblẽa nu le ʋuka suesuesue siwo le ayikuawo me ŋu. Wole abe lãgbalẽgoe siwo le tsilénu me ene. Dɔléle bubu siwo dzena le hatsotso sia me dometɔ aɖewoe nye:

• Ayiku me amyloidosis ƒe dɔléle. Esia lɔ ƒomevi siwo woyɔna be AL amyloidosis, AH amyloidosis, kple AHL amyloidosis ɖe eme.
• Cryoglobulinemic glomerulonephritis (CG) ƒe dɔléle.
• Fibrillary glomerulopathy (FGN) ƒe dɔléle si nye fibrillary glomerulopathy.
• Dɔlélenutsiŋutete ƒe glomerulopathy (ITG).
• Glomerular dɔléle siwo do ƒome kple monoclonal proteins. Esia lɔ nɔnɔme siwo woyɔna be `kɔsɔkɔsɔ bɔbɔe` kple `kɔsɔkɔsɔ kpekpewo ƒe ƒuƒu ƒe dɔlélewo` ɖe eme.

2. Tubulointerstitial dɔlélewo

Dɔléle siawo gblẽa nu le ʋukawo (tubules) siwo tsɔa nunyiame kple tsi siwo ayikuwo ɖena la trɔna yia ʋua me, kple lãkusi siwo ƒo xlã wo ŋu. Kpɔɖeŋu aɖewoe nye:

• Fanconi ƒe kɔsɔkɔsɔ si me kɔ (LCFS).
• Light chain proximal tubulopathy (LCPT) si me kristalo mele o.
• Histiocytosis si dzraa kristalo ɖo.

3. Nɔnɔme siwo gblẽa nu le ʋuka siwo le ayiku me ŋu (Intra-renal vascular lesions) .

Nɔnɔme siawo gblẽa nu le ʋuka siwo le ayiku me ŋu. Kpɔɖeŋu aɖee nye ʋumenugbagbeviɣidɔ si mebɔ o (aHUS) . Esiae nye esime ʋuka suesuesue siwo le ayikuwo me xea mɔ na ʋu ƒe sisi.

Aleke gbegbee MGRS bɔe?

Le nyateƒe me la, MGRS nye nɔnɔme si mebɔ kura o . Wobu akɔnta be egblẽa nu le ame siwo mede 1% o ŋu. Ebɔ ɖe amesiwo xɔ wu ƒe 60 koŋ me .

Nukae nye MGRS ƒe dzesiwo?

MGRS ƒe dzesiwo ate ŋu ato vovo le MGRS ƒomevi si le ŋuwò nu. Dzesi siwo bɔ dometɔ aɖewoe nye:

• Ðeɖiteameŋu ƒe seselelãme ŋutɔ (ɖeɖiteameŋu) .
• Afɔ siwo le ete, afɔkpodzi, alo afɔwo ƒe vuvu . Ɣeaɖewoɣi la, dɔgbo, asiwo, alo mo hã ate ŋu avuvu.
• Nephrotic syndrome dzɔna. Esia ƒe dzesi enye protein ƒe dodo le aɖuɖɔ me kple ʋumemi ƒe agbɔsɔsɔ si dzina ɖe ʋu me.
• Aɖuɖɔ si me futukpɔ alo adzalẽ le . Esia ate ŋu anye protein si le aɖuɖɔ me ƒe dzesi (proteinuria).
• Aɖuɖɔtoe enuenue nye alesi wòhiã be nàɖe aɖuɖɔ enuenu.
• Ʋu si le aɖuɖɔ me (hematuria) alo aɖuɖɔ si trɔna zua pink.
• Aɖuɖɔtoe kple ʋuʋudedi.
• POEMS ƒe dɔléle . Esia hã nye nɔnɔme si mebɔ o.
• Afɔ kple afɔwo ƒe ʋuʋu, ʋuʋudedi, alo gbɔdzɔgbɔdzɔ (esiawo nye ahɔhɔ̃mekawo ƒe dɔléle ƒe dzesiwo).
• Ŋutilã ƒe amadede ƒe tɔtrɔ (teƒe siwo me kɔ alo esiwo do viviti).
• Yagbɔgbɔ sesẽ (gbɔgbɔtsixetsixe).

Nukae hea MGRS vɛ?

Nu vevitɔ si gbɔ MGRS tsonae nye ayiku ƒe gbegblẽ si M protein siwo míeyɔ va yi hena vɛ. M proteins nye dɔlékuiwutike ƒomevi aɖe si mewɔa dɔ nyuie o, eye wowɔe le mɔ gbegblẽ nu. Wotsɔa ʋumenugbagbevi dzĩwo, ʋumenugbagbevi ɣi ƒomevi aɖe, alo B lãmenugbagbeviwo ƒe nɔnɔmetata siwo gblẽ (kɔpiwo) wɔa woe.

Ame aɖewo ate ŋu akpɔ ʋumenugbagbevi dzĩwo ƒe nɔnɔmetata siawo siwo awɔ M proteinwo evɔ womagblẽ nu le ŋutinu aɖeke ŋu o. Woyɔa nɔnɔme sia be Monoclonal Gammopathy of Undetermined Significance (MGUS) . Gake le amesiwo ŋu MGRS le gome la, M protein siawo ƒoa ƒu ɖe ayiku me eye wogblẽa nu le wo ŋu.

Nuka koŋue hea MGRS vɛ?

Eŋutinunyalawo menya nusi tututu hea MGRS vɛ haɖe o. Wosusu be dɔlélenutsiŋutete ƒe dɔlélewo , domenyiŋusẽfianu ƒe tɔtrɔ , dɔlékuiwo, kple nutoa me ƒe nɔnɔmewo katã gbɔe wòtso.

Kuxi kawoe ate ŋu ado mo ɖa le MGRS ta?

Plasma cell clones siawo kple MGRS ate ŋu agblẽ nu le ayiku ŋu, si ate ŋu ahe kuxi sesẽwo vɛ. Esiawo dometɔ aɖewoe nye:

• Ayikudɔ si nɔa anyi didi (CKD) .
• Ayiku (ayiku) ƒe dɔléle si le eƒe nuwuwu .
• Ayiku ƒe dɔmawɔmawɔ nyuie .
• Tsitsi va zu dɔléle bubuwo. Le ame aɖewo gome la, plasma cell clones ƒe xexlẽme dzina ɖe edzi ale gbegbe be ate ŋu ava zu ʋumenugbagbevi ɣiwo ƒe kansa ƒomevi aɖe si woyɔna be multiple myeloma (MM) . MGRS ate ŋu ava zu kansa bubuwo hã, abe Waldenström macroglobulinemia , ʋumenugbagbeviɣidɔ si nɔa anyi didi (CLL) , alo B-cell lymphoma .

Aleke wowɔa dea dzesi MGRS?

Ðɔkta ate ŋu aɖo kpe MGRS dzi to ayiku dodokpɔ wɔwɔ me . Esia bia be nàɖe lãkusi sue aɖe le wò ayiku me ahadoe kpɔ be M protein le eme hã. Woate ŋu ado wò ʋu, aɖuɖɔ, alo ƒumeŋuɖui ƒe kpɔɖeŋuwo hã kpɔ.

Ðe atike aɖe li na MGRS?

Ẽ, atike aɖewo li na MGRS. Ðɔktawo tsɔa atike siwo ɖi atike siwo wozãna tsɔ daa kansae la daae. Atike siawo wua lãmenugbagbevi siwo ŋu kuxia le alo xea mɔ na wo be womegawɔa kɔpi geɖe o. Atike bubuwo ate ŋu akpe ɖe ame ŋu be wòaɖe abi dzi akpɔtɔ. Taɖodzinu vevitɔ si le atikewɔwɔ ŋue nye be wòana wò ayikuwo nawɔ dɔ eye woaxe mɔ na wo be woagagblẽ o.

Le MGRS ƒe nɔnɔme aɖewo me la, ɖɔktawo ate ŋu ado lãmenugbagbeviwo na ame bubuwo hã . Esia tsɔa lãmenugbagbevi siwo le lãmesẽ me ɖɔlia wò lãmenugbagbevi siwo gblẽ. Ne wò ayiku gblẽ vevie la, ate ŋu ahiã hã be woado ayiku na wò alo ado ayiku na wò .

Atike kple atike tɔxɛ siwo wozãna na MGRS

Atike si ƒomevi woana wò anɔ te ɖe MGRS ƒomevi si le asiwò dzi. Atikewɔwɔ ƒe mɔnu aɖewoe nye esi:

• Dɔlékuiwutike siwo nye monoclonal - e.g. rituximab, atike si nye daratumumab.
• Proteasome mɔxenuwo - e.g. bortezomib, carfilzomib, atike si woyɔna be ixazomib.
• Dɔlélenutsiŋutete ƒe atikewo - e.g. thalidomide, lenalidomide, atike si wotsɔna ƒoa ƒui.
• Alkylating-nuwo - e.g. cyclophosphamide, bendamustine, lãgbalẽ si woyɔna be melphalan.
• Corticosteroids - e.g. prednisone, si nye dexamethasone ƒe atike.
• Autologous stem cell ƒe dodo na ame.

Nukawoe nye atikewɔwɔa ƒe nugbegblẽ le ame ŋu?

Atikewɔwɔ abe atikewɔmɔnu ene ate ŋu ahe nugbegblẽ le ame ŋu geɖe siwo medzea ame ŋu o vɛ, eye ɣeaɖewoɣi hã ate ŋu agblẽ nu le ŋutinuwo ŋu. Esia tae ɖɔktawo tiaa atikewɔmɔnuwo nyuie ŋutɔ be woakpɔ viɖe geɖe tso wo me eye woaɖe afɔku si le nugbegblẽ le ame ŋu sesẽwo me dzi akpɔtɔ. Nusiwo ate ŋu ado tso eme dometɔ aɖewoe nye:

• Ʋusɔgbɔdɔ ( ʋu ƒe veve).
• Ʋu si le dodom.
• Miléle.
• Dɔmetɔtrɔ.
• Ɖeɖi te eŋu.
• Dɔlékui, dɔlékui kple fungi ƒe dɔlékuiwo ƒe afɔku si dzina ɖe edzi.
• Nuɖuɖua mevivina o.
• Aɖuɖɔtoe kple ʋuʋudedi.

Ðe woate ŋu axe mɔ ɖe MGRS nua?

Esi míenya nusi tututu hea MGRS vɛ o ta la, mɔ aɖeke meli koŋ si dzi míato axe mɔ ɖe enu fifia o.

Nukae nàte ŋu akpɔ mɔ na ne MGRS le ŋuwò?

Togbɔ be MGRS nye dɔléle si woate ŋu ada hã la, zi geɖe la, medzona keŋkeŋ o. Ate ŋu agadzɔ ake le atikewɔwɔ vɔ megbe. Ne wò ayikuwo gblẽ vevie la, ahiã be woado ayiku na wò.

Ame siwo ŋu MGRS le dometɔ siwo ade 18% ava xɔ kansa si woyɔna be multiple myeloma emegbe. Eyata wò ɖɔkta atsɔ ʋu kple aɖuɖɔtoe alé ŋku ɖe ŋuwò edziedzi, elabena woate ŋu ade dzesi esia kaba ne edzɔ.

Ɣeyiɣi didi kae nàte ŋu anɔ agbe kple MGRS?

Ɣeyiɣi didi si nàte ŋu anɔ agbe kple MGRS nɔ te ɖe MGRS ƒomevi si le ŋuwò, eƒe sesẽme, kple alesi nèwɔa nu ɖe ​​atikewɔwɔ ŋui dzi. Amesiwo ƒe ayiku megblẽ boo o ƒe nukpɔsusu nyo wu amesiwo ƒe ayiku gblẽ vevie wu. Le kpɔɖeŋu me, le numekuku aɖe me la, amesiwo ŋu amyloidosis si do ƒome kple MGRS le la nɔa agbe abe ƒe 10 ene le mamã dedie nu le dɔlélea kpɔkpɔ vɔ megbe. Gake amesiwo si amyloidosis mele o la nɔa agbe didi wu sã. Azɔ hã, ame siwo wɔa nu ɖe ​​atikewɔwɔ ŋu nyuie lae kpɔa nu nyuitɔ kekeake.

Aleke malé be na ɖokuinye?

Kpɔ egbɔ be yewɔ ɖe aɖaŋuɖoɖo ɖesiaɖe si wò ɖɔkta aɖo na wò le wò ayiku ƒe lãmesẽ dzi kpɔkpɔ le aƒeme dzi. Esia ate ŋu alɔ nusiwo le abe aha sesẽ ene dzi ɖeɖe kpɔtɔ, kple nusiwo ate ŋu ana ayikudɔléle, abe ʋusɔgbɔdɔ kple suklidɔ ene, nate ŋu aɖu dzi. Togbɔ be nu siawo mate ŋu ada MGRS tẽ o hã la, woate ŋu akpe ɖe ame ŋu be ayiku ƒe gbegblẽ geɖe wu ƒe afɔkua dzi naɖe akpɔtɔ. Wò ɖɔkta ate ŋu akpe ɖe ŋuwò hã be nàɖu atikewɔwɔ ƒe nugbegblẽ le ame ŋu dzi.

Ɣekaɣie wòle be makpɔ ɖɔkta?

Ne ayiku me kuxi aɖe ƒe dzesiwo le ŋuwò, abe aɖuɖɔtoe si me futukpɔ alo pink le, aɖuɖɔɖɔɖɔ enuenu, alo aɖuɖɔtoe sue aɖe ko ene la, kpɔ egbɔ be yeyi ɖɔkta gbɔ.

Nya kawoe wòle be mabia nye ɖɔkta?

Anyo be nàbia nya siawo wò ɖɔkta:

• Atikewɔwɔ kawoe li na MGRS ƒomevi si le ŋunye?
• Nukae mate ŋu awɔ be ayiku nagagblẽ o?
• Dzesi yeye alo esiwo le gbegblẽm ɖe edzi kawo ŋue wòle be malé ŋku ɖo?

Mlɔeba la, nu vevitɔ kekeake (Take-Home Message) .

MGRS nye dɔléle sesẽ aɖe si ate ŋu agblẽ nu le ayikuwo ŋu le mɔ si ate ŋu ade ame ƒe agbe afɔku me nu. Gake ɖo ŋku edzi be menye amesiame si ŋu MGRS le lae axɔ kansa o. Wò ɖɔkta alé ŋku ɖe ŋuwò edziedzi eye wòaɖe afɔ aɖewo atsɔ ade dzesi kuxi yeye ɖesiaɖe ahadae kaba ale si wòanya wɔ . Fifia dɔdamɔnu yeyewo na wòte ŋu na wò agbenɔnɔ nanyo ɖe edzi eye wòɖe dɔléle sesẽwo xɔxɔ ƒe afɔkua dzi kpɔtɔ. Eyata mègavɔ̃ o, wɔ ɖe wò ɖɔkta ƒe aɖaŋuɖoɖo dzi, eye nàdze ŋgɔ nɔnɔme sia.

` MGRS, Monoclonal Gammopathy of Renal Significance, Ayikudɔléle, M Proteins, Plasma Lãmenugbagbeviwo, Ayiku ƒe Biopsy, Nephrotic Syndrome, Multiple Myeloma