Ðe nèkpɔa nulɔdɔ zi geɖe eye teƒe dzĩwo kple ʋuʋudedi le wò ŋutilã katã ŋua? Esia ate ŋu anye Mastocytosis!

Ðe wò ŋutilã biãna, ʋuʋudedi, eye ɣeaɖewoɣi la, ʋuʋudedi sesẽna nɔa ŋuwò zi geɖea? Alo ɖe nu suetɔ kekeake gɔ̃ hã ƒe nulɔdɔ sesẽ aɖe le fu ɖem na wò ŋkeke geɖea? Àte ŋu asusu be, "Nye nulɔdɔ bubu aɖe le fu ɖem nam." Gake ɣeaɖewoɣi le dzesi siawo megbe la, nɔnɔme aɖe si mebɔ o ate ŋu anɔ anyi si ŋu míese nu tsoe haɖe o. Nɔnɔme mawo dometɔ ɖekae nye `(Mastocytosis)`. Togbɔ be ŋkɔa aɖi nusi wɔ nuku vie hã la, mina míaƒo nu tso eŋu bɔbɔe.

Nukae nye Mastocytosis? Kpuie ko la,...

Bu eŋu kpɔ, lãmenugbagbevi siwo le abe dedienɔnɔdzikpɔla suewo ene le míaƒe ŋutilã me. Míeyɔa wo be ``mast cells.`` ``Mast cells`` siawo nye míaƒe ``immune system.`` ƒe akpa aɖe.`` Wole abe míaƒe dukɔa ƒe dedienɔnɔ dɔwɔlawo ene. Ne futɔwo abe dɔlékuiwo, dɔlékuiwo, kple nusiwo nana ŋutilãa melɔ̃na o ge ɖe ŋutilã me la, ``mast cells`` siawo dona ɖe ŋgɔ hewɔa avu.

Ne `(mast cells)` siawo wɔ nu ɖe ​​futɔwo ŋu la, woɖea atike aɖe si woyɔna be `(histamine) dona.` `(histamine)` siae hea nulɔdɔ ƒe dzesiwo vɛ le míaƒe ŋutilã me (le kpɔɖeŋu me, ʋuʋudedi, ŋutigbalẽ ƒe biã, vuvu). `(mast cells)` siawo hã kpena ɖe acid wɔwɔ ŋu le dɔgboa me.

Zi geɖe la, `(mast cells)` siawo wɔa woƒe dɔ eye woɖua futɔwo dzi eye emegbe wogazia ɖoɖoe ake. Ke hã, nusi dzɔna le amesi ŋu `(Mastocytosis)` le ƒe ŋutilã me enye be `(mast cells)` siawo dzina ɖe edzi le mɔ si mesɔ o nu eye womate ŋu aɖu wo dzi o. Ne wodzi ɖe edzi alea la, lãmenugbagbevi siawo ɖea `(histamine) doa go fũ akpa.` Ɣemaɣie nèxɔa nulɔdɔ gã aɖe ɖe nu suetɔ kekeake gɔ̃ hã ŋu, alo ne nulɔdɔ dze ŋuwò la, evɔ̃ɖi wu le ame dzɔdzɔe gome, eye wònɔa anyi didi wu.

Vevietɔ wu la, amesiame si ŋu Mastocytosis le la le afɔku me wu be wòaxɔ anaphylaxis , si nye nulɔdɔ si dzɔna kpata, si nu sẽ, eye ate ŋu ade eƒe agbe afɔku me . Eya ta ele vevie be míanya esia.

Togbɔ be ɖɔktawo mate ŋu ada Mastocytosis keŋkeŋ o hã la, woate ŋu aɖu dzesiawo dzi ahada kuxi ɖesiaɖe si ate ŋu ado mo ɖa.

Nukawoe nye Mastocytosis ƒomevi veviwo?

Dɔléle sia ƒomevi vevi eve li:

1. Cutaneous Mastocytosis: Zi geɖe la, esia dzɔna le vidzĩwo kple ɖevi suewo ŋu . Ke hã, ate ŋu adzɔ le ame tsitsiwo hã gome. Nusi dzɔna le afisia enye be ``mast cells`` siwo mesɔ o ƒoa ƒu ɖe ŋutigbalẽa me, si wɔnɛ be ʋuʋudedi kple teƒe siwo ƒe amadede nye dzĩ, dzĩ dzẽ, blɔ, alo amadede vovovowo. Ðeko wòle abe ɖevi sue aɖe ƒe ŋutilã ŋu zi ɖeka eye woƒoa wo madzudzɔmadzudzɔe ene.

2. Systemic Mastocytosis: Ame tsitsiwo mee wokpɔa esia ƒomevi wu.Le afisia la, ``mast cells`` siwo mesɔ o ƒoa ƒu ɖe ememe ŋutinuwo abe ƒumeŋuɖui, ƒuwo, míaƒe nuɖuɖumeŋusẽ, kple lãkusiwo me. Bu eŋu kpɔ, ne lãmenugbagbevi siawo ƒo ƒu ɖe ƒumeŋuɖui me wògbɔ eme la, ate ŋu akpɔ ŋusẽ ɖe míaƒe ʋumenugbagbeviwo ƒe wɔwɔme dzi. Azɔ hã, ne lãmenugbagbevi siawo dzi ɖe edzi le dɔgboa me la, woate ŋu awɔ dɔgboa me aɖi si sɔ gbɔ akpa eye wòana dɔgboa naxɔ abi.

Ðe esia nye dɔléle si bɔa?

Ao, le nyateƒe me la, esia nye nɔnɔme si mebɔ kura o. Le numekukuwo nu la, wogblɔ be ame ɖeka le ame akpe ewo (10,000) me alo ame ɖeka le akpe blaeve (20,000) ɖesiaɖe me koe dɔléle sia le. Eyata esɔ be menye amesiamee nya nu tso eŋu o.

Nukawoe nye Mastocytosis ƒe dzesi siwo ate ŋu anɔ ame ŋu?

Ne ``mast cells`` siawo le dzidzim ɖe edzi le mɔ si mesɔ o nu la, dzesi vovovowo ateŋu ado mo ɖa. Togbɔ be dzesi siawo ate ŋu asẽ ŋutɔ le ame aɖewo gome hã la, woate ŋu asẽ le ame bubuwo gome.

• Ƒu ƒe vevesese: Sese le ɖokuiwò me abe ɖe wò ƒuwo le veve sem ene.
• Aɖuɖɔtoe (dɔgbo ƒe veve): Ele be nàyi tsileƒe enuenu.
• Alɔ̃madɔmadɔ (abe susumanɔamesi ene): Kasia ŋkuwo trɔna zua blɔ eye wòdzena abe ɖe wò susu megale dɔ wɔm o ene.
• Dzimaɖitsitsi kple vɔvɔ̃ ƒe nɔnɔme si nɔa ame me ɣesiaɣi: Dzimaɖitsitsi ƒe seselelãme le susu aɖeke manɔmee.
• Ʋuʋudedi: Ŋutilãa ƒe amadede biã zi ɖeka le ŋutilã bliboa me.
• Aɖuɖɔtoe alo teƒe siwo le ŋutigbalẽa ŋu: Abe alesi míeƒo nu tso eŋu va yi ene.
• Taɖuame: Taɖuame enuenu.
• Taɖuame si tsoa ʋu ƒe sisi gbɔ: Taɖuame ƒe seselelãme ne ètsi tre tso tsitrenu.
• Ƒunukpeƒeveame: Ƒunukpeƒeveame.
• Teƒe dzĩ alo aŋutiɖiɖi le ŋutigbalẽa ŋu: Esiawo ate ŋu anye ʋuʋudedi alo womaƒoe o.

Ƒe nenie Mastocytosis dzea egɔme tsoe?

Etoa vovo le dɔléle ƒomevi si le eŋu nu. Le nyateƒe me la,:

• Cutaneous Mastocytosis: Esia ate ŋu adze vidzĩ siwo wodzi yeyee gɔ̃ hã dzi.
• Systemic Mastocytosis: Zi geɖe la, edzea ame tsitsi siwo xɔ wu ƒe 50 dzi.

Nuka tae wòle alea ɖo?

`(Mastocytosis)` menye nusi wodzi mí kplii o. Etsoa domenyiŋusẽfianu siwo woyɔna be `(KIT genes)` ƒe tɔtrɔ gbɔ. `(KIT domenyiŋusẽfianu)` siawo nye domenyiŋusẽfianu siwo kpena ɖe lãmenugbagbevi ƒomevi aɖewo ŋu, abe míaƒe ʋumenugbagbeviwo, `(mast cells)` ene, tsina. Le `(Mastocytosis) me la, `(KIT domenyiŋusẽfianu)` siawo siwo trɔ la naa dzesi si mesɔ o `(mast cells)` be "woadzi ɖe edzi akpa eye woatsi le mɔ si dzi womate ŋu aɖu o nu."

Nukae nye nusiwo hea mastocytosis vɛ?

Esia ate ŋu ato vovo tso ame ɖeka gbɔ yi bubu gbɔ. Menye amesiamee ƒe nusiwo ʋãa ame la sɔ o. Gake nu aɖewo li siwo ate ŋu ana dzesiawo nadzi ɖe edzi zi geɖe:

• Ŋutilã tutu alo etutu: Esia ate ŋu adzɔ ɖe ame aɖewo hã dzi ne wodo awu sesẽwo ko.
• Kamedede kple kamedede si gbɔ eme.
• Nudzodzoewo ƒe ƒoƒo alo woƒe ɖuɖu:Vevietɔ lãwo abe anyidiwo, ʋetsuviwo, kple anyiwo ene.
• Ahanono nono.
• Nuɖuɖu aɖewo: Vevietɔ nuɖuɖu siwo me nu ʋeʋĩwo le, siwo me wode nuɖuɖuvivinuwoe ŋutɔ.
• Atike aɖewo: Le kpɔɖeŋu me, vevesesenutsitike siwo woyɔna be ``NSAIDs`` (Nonsteroidal anti-inflammatory drugs), lãmekawo ƒe gbɔdzɔe, kple atike siwo naa alɔ̃dɔdɔ ``(anesthesia)``.
• Dzoxɔxɔ ƒe tɔtrɔ kpata: Abe titri yi tsi fafɛ ŋutɔ me ene.
• Ŋutilã alo susu me nuteɖeamedzi: Zi geɖe la, esia kpɔa ŋusẽ gã aɖe ɖe amewo dzi.

Nukawoe nye kuxi siwo ate ŋu ado tso Mastocytosis me?

Dɔléle sia ate ŋu ahe lãmesẽkuxi bubuwo vɛ, siwo dometɔ aɖewoe nye:

• Ƒumeŋuɖui: Esia nana ƒuwo gbɔdzɔna eye wogbãna bɔbɔe.
• Aklã me kuxiwo: Nɔnɔme si me aklã vuvu eye megawɔa dɔ nyuie o.
• Cytopenia: Ŋutilã ƒe ŋutete be wòawɔ ʋumenugbagbevi siwo sɔ.
• Dɔlékuiwo xɔxɔ enuenu.

Aleke wowɔa dea dzesi Mastocytosis?

Wò ɖɔkta abia wò dzesiwo eye wòado wò ŋutigbalẽ kpɔ. Tsɔ kpe ɖe eŋu la, woate ŋu awɔ dodokpɔwo abe:

• Ʋudodokpɔ: Kpɔ enzyme si woyɔna be ``tryptase`` ƒe agbɔsɔsɔ le wò ʋu me ɖa. ``Tryptase`` sia nye nane si ``mast cells`` ɖea asi le ne wowɔ nu ɖe ​​futɔwo ŋu.
• Ƒuwo me toto: Be woakpɔe ɖa be ƒuwo gblẽ hã.
• Ƒumeŋuɖui ƒe lãmenugbagbeviwo ɖeɖe: Woɖea ƒumeŋuɖui ƒe kpɔɖeŋu aɖe hekpɔa ƒumeŋuɖui siwo mesɔ o hã.
• Domenyiŋusẽfianu dodokpɔwo: Ne Systemic Mastocytosis le ŋuwò la, woate ŋu ado wò kpɔ be tɔtrɔ aɖewo le KIT domenyiŋusẽfianuwo me hã.

Nukawoe nye Mastocytosis ƒe atikewo?

Atikewɔwɔ ƒe tiatia vovovowo le dɔlélea ƒomevi kple wò dzesiwo nu. Zi geɖe la, ɖɔktawo zãa atikewo kple atike bubuwo tsɔ ɖea dzesiawo dzi kpɔtɔna.

Atike ƒomevi siwo wozãna na Mastocytosis:

• Antacids na dɔgbo ƒe kuxiwo (Antacids - H2 blockers): Na nuwo abe dɔgbo ƒe dɔdzẽ kple dɔgbo ƒe dɔléle ene.
• Antihistamines: Ðe ŋutigbalẽ ƒe dzesi siwo me nulɔdɔ le abe ʋuʋudedi kple dzodzro ene dzi kpɔtɔna.
• Bisphosphonates na ƒumeŋuɖui: Do ŋusẽ ƒuwo.
• Corticosteroids: Ðea ʋuʋudedi dzi kpɔtɔna.
• Dɔlékuiwutike siwo nye monoclonal: vevietɔ atikewo abe omalizumab (Xolair®) ene.
• Vevesesenutsitikewo .

Atikewɔwɔ bubuwo:

• Ultraviolet (UV) kekeli ƒe atikewɔwɔ: Ðe teƒe siwo do viviti le ŋutigbalẽa ŋu dzi kpɔtɔ.
• Atikewɔwɔ si ŋu woɖo taɖodzinu ɖo .
• Epinephrine na anaphylaxis:Atikewɔwɔ kpata na nulɔdɔ sesẽ aɖe.
• Atikewɔwɔ: Esia mebɔ kura o eye ne Mastocytosis trɔ zu kansa ko hafi wonaae.
• Allogeneic stem cell (ƒumeŋuɖui) dodo na ame: Wobua esia hã le mastocytosis ƒe nɔnɔme sesẽ ŋutɔ, siwo sesẽ me. Ame akpa gãtɔ mehiã esia o.

Ðe kuxi aɖewo alo nugbegblẽ le ame ŋu le atikewɔwɔa mea?

Ẽ, abe alesi wòle le atikewɔwɔ ɖesiaɖe gome ene la, nugbegblẽ aɖewo li siwo ate ŋu ado mo ɖa le Mastocytosis ƒe atikewɔwɔwo me. Ðewo ate ŋu anye nu suewo, evɔ bubuwo ya ate ŋu anye esiwo metsɔ ɖeke le eme o. Ne Mastocytosis le ŋuwò la, wò ɖɔkta aɖe atike siwo wɔm nèle kple nusiwo ate ŋu ado tso eme la me na wò.

Ðe woate ŋu ada Mastocytosis keŋkeŋa?

Le nyateƒe me la, wobua Mastocytosis be enye dɔléle si womate ŋu ada keŋkeŋ o, gake woate ŋu akpɔ egbɔ to dzesiwo dzi ɖuɖu kple kuxiwo dada me .

Atike ɖeka kolia si ateŋu ada esiae nye ``allogeneic stem cell (ƒumeŋuɖui) dodo'' si míeyɔ va yi. Gake dɔnɔ siwo ŋu dɔlélea nu sẽ wu eye wòsesẽ wu koe wokafu ema gɔ̃ hã na.

Ðe Mastocytosis nu sẽna ɖe edzi le ɣeyiɣi aɖe megbea?

Ne womewɔ atike nɛ o la, dzesiawo ate ŋu agblẽ ɖe edzi le ɣeyiɣi aɖe megbe, vevietɔ le amesi ŋu systemic mastocytosis , le gome, elabena mast cells siwo mesɔ o yi edzi le ŋusẽ kpɔm ɖe ƒumeŋuɖui kple ŋutinu ememetɔwo dzi.

Ðe woate ŋu axe mɔ ɖe Mastocytosis nua?

Nublanuitɔe la, ao. Esi wònye be KIT domenyiŋusẽfianu ƒe tɔtrɔ gbɔe wòtsona ta la, mɔ aɖeke meli si dzi woato axe mɔ ɖe enu o. Gake ne Mastocytosis le ŋuwò la, àte ŋu aɖu wò dzesiwo dzi ne ènya nusi tututu hea wò nulɔdɔwo vɛ eye nèƒo asa na wo alesi nàte ŋui.

Le kpɔɖeŋu me, ne nusi ʋãa atike aɖewo le asiwò la, anyo be nàdo atikewɔwɔ ŋuti nuxlɔ̃ame asigɛ si dzi woŋlɔ wo ɖo. Esia ana ame bubuwo nanya atike siwo mele be nàno ne nɔnɔme kpata aɖe do mo ɖa o.

Ne Systemic Mastocytosis le ŋuwò la, ele vevie be nàtsɔ atikekui si wodona na ɖokuiwò si me epinephrine le la ɖe asi ɣesiaɣi. Woate ŋu azã esia atsɔ ada nulɔdɔ sesẽ siwo ate ŋu ade ame ƒe agbe afɔku me abe anaphylactic shock ene.

Aleke malé be na ɖokuinye? (Mastocytosis) Esime wònɔ agbe kple...

Esi wònye be Mastocytosis nye dɔléle si womate ŋu ada keŋkeŋ o ta la, ɖewohĩ ahiã be nànɔ agbe kplii hena ɣeyiɣi aɖe. Ate ŋu asesẽ. Ke hã, nu aɖewo siwo ate ŋu akpe ɖe ŋuwòe nye esi:

• Nya nusi tututu hea wò dzesiawo vɛ.Nusiwo ʋãa ame sia ame to vovo. Àdi be yeawɔ ŋkekenyuigbalẽ eye nàŋlɔ ɣeyiɣi si me wò dzesiawo nu sẽ ɖe edzi, nusiwo nèɖu kple nunono, kple nusi nèwɔ ɣemaɣi.
• Nuteɖeamedzi ate ŋu ana dzesi siawo nagado gã ɖe edzi. Ne èhiã kpekpeɖeŋu le nuteɖeamedzi dzi ɖuɖu me la, bia wò ɖɔkta tso nuteɖeamedzi dzi ɖuɖu ƒe ɖoɖowo ŋu. Nuwo abe ŋugbledede kple yoga ene ate ŋu akpe ɖe ŋuwò.
• Do asigɛ si naa atikewɔlawo ƒe nuxlɔ̃ame. Ele be wòaŋlɔ wò atikewɔwɔ ƒe nɔnɔme kple atike ɖesiaɖe si mele be nàno o.
• Tsɔ epinephrine auto-injector aŋe ɖe asi ɣesiaɣi. Ate ŋu axɔ ame ɖe agbe ne nulɔdɔ sesẽ aɖe do mo ɖa.

Ɣekaɣie wòle be makpɔ nye ɖɔkta?

Àdo wò ɖɔkta kpɔ edziedzi . Woalé ŋku ɖe wò dzesiwo ŋu eye woatrɔ wò atikewɔwɔ ne ehiã. Ne dzesi yeyewo va dze ŋuwò, alo ne wò dzesiawo gagblẽ ɖe edzi la, gblɔe na wò ɖɔkta enumake.

Ɣekaɣie wòle be mayi Dɔwɔƒe si Kpɔa Nudzɔdzɔ Kpatawo Gbɔ?

Ne nulɔdɔ sesẽ aɖe ŋutɔ le ŋuwò si dzi womate ŋu atsɔ atikewo aɖu o la, yi xɔ si me wowɔa dɔ kpata le enumake. Eyae nye be ne èkpɔ dzesiwo abe gbɔgbɔ sesẽ, ŋɔti me ƒoƒo, taɖuame sesẽ, alo susu ƒe dɔmawɔmawɔ nyuie ene.

Nya kawoe wòle be mabia nye ɖɔkta?

Ne Mastocytosis le ŋuwò la, anyo be nàbia wò ɖɔkta nya siawo abe esiawo ene:

• Mastocytosis ƒomevi ka ƒomevie le ŋunye?
• Atikewɔwɔ kawoe li na amesi ƒomevi le asinye?
• Ðe atikewɔwɔa aɖe nye dzesiawo ɖa keŋkeŋa?
• Dzesi bubu kawoe ate ŋu afia be nye nɔnɔmea le sesẽm ɖe edzi?
• Ðe wòahiã be mawɔ atike tegbeea?
• Ne dɔléle aɖe si nu sẽ wu le ŋunye la, nukae nye nye ŋgɔdonya?
• Ðe dɔdamɔnu dodokpɔ aɖewo li siwo me mate ŋu akpɔ gome lea?

Mlɔeba la, nusi dzi nàɖo ŋkui (Take-Home Message) .

Mastocytosis nye nɔnɔme si mebɔ o si nana mast cells ƒe tsitsi si mesɔ o, si dzi womeɖu o le ŋutilãa me. Ate ŋu agblẽ nu le ŋutigbalẽ kple ememe ŋutinuwo ŋu. Ame akpa gãtɔ ate ŋu akpɔ woƒe dzesiwo gbɔ to dzesidede nusiwo hea dɔlélea vɛ kple wo ƒoƒo asa na wo kpakple atikewo nono me. Ele vevie hã be nàna epinephrine-tike nanɔ asiwò be nàtsɔ ada nusiwo ŋu nulɔdɔ sesẽwo le.

Togbɔ be esia nye dɔléle si womate ŋu ada keŋkeŋ o hã la, ne èɖo aɖaŋu na wò nyuie le atikewɔlawo kple atikewɔwɔ me la, àte ŋu adze agbagba aɖu dzesiawo dzi ahanɔ agbe si sɔ. Ne nyabiase bubu aɖewo gale asiwò ku ɖe esia ŋu la, mègahe ɖe megbe le nuƒoƒo kple wò ɖɔkta me o. Woakpe ɖe ŋuwò.

` Mastocytosis, mast cells, histamine, nulɔdɔ, ŋutigbalẽdɔlélewo, dɔlélenutsiŋutete, anaphylaxis