Ðe nèsea veve le mɔ wɔnuku aɖe nu zi geɖea? Mina míasrɔ̃ nu geɖe tso (Systemic Mastocytosis) sia ŋu!

Ðe teƒe biã siwo le dzo dam le wò ŋutigbalẽ ŋu ɣeaɖewoɣia? Alo ɖe dɔgbo le vevem kple taɖuame si va nɔa yiyim ko? Ne nusiawo dzɔ enuenu la, ateŋu anye nɔnɔme si mebɔ o si woyɔna be ``Systemic Mastocytosis'' si ŋu míele nu ƒom tsoe egbea. Mègatsi dzi o, esia menye dɔléle si dzea ame geɖe dzi o. Gake ele vevie be nànya esia.

Eyata nukae nye ``Systemic Mastocytosis'' sia?

Kpuie ko la, `(Systemic Mastocytosis)` nye dɔléle si mebɔ o si do ƒome kple míaƒe ʋu. Nusi dzɔna le esia me enye be lãmenugbagbevi tɔxɛ aɖe si woyɔna be `(Mast cells)` le míaƒe ŋutilã me tsina le mɔ si mesɔ o nu. Bu eŋu kpɔ, lãmenugbagbevi siawo le abe asrafo suewo ene le míaƒe ŋutilã me. Woawoe nye amesiwo, abe míaƒe dɔlélenutsiŋutete ƒe akpa aɖe ene, dea dzesi dutatɔwo ƒe amedzidzelawo (abe dɔlékuiwo, nusiwo nana ŋutilã melɔ̃a nu o, kple bubuawo ene) hewɔa avu kpli wo. Ne dutatɔ sia tɔgbe va la, mast cell siawo ɖea atikewo abe `(Histamine)`. `(Histamine)` siae hea nulɔdɔ ƒe dzesi mawo vɛ (abe dzodzro, ʋuʋudedi, vuvu ene). Zi geɖe la, mast cells dzudzɔa `(Histamine)` sia dodo ne wowu dɔa nu. Ke hã, le amesi ŋu `(Systemic Mastocytosis)` le ƒe ŋutilã me la, mast cell siawo siwo mesɔ o la tsina akpa eye woɖea `(Histamine)` ɖe go ɣesiaɣi. Ðeko wòle abe ame aɖe gblẽ tsi ƒe pɔmpi aɖe ɖi eye emegbe wòŋlɔ be be yeatsii ene. Esia tae dzesi siwo le abe nulɔdɔ ene nɔa mía ŋu. Menye míaƒe ŋutigbalẽ koe ate ŋu agblẽ nu le o, ke ate ŋu agblẽ nu le míaƒe ŋutinu ememetɔwo abe míaƒe aklã, dɔgbo, kple dɔgbowo ene hã ŋu. Wogblɔ be nɔnɔme sia si woyɔna be ``Systemic Mastocytosis,'' la dzea ame 13 ko dzi le ame 100,000 ɖesiaɖe me le xexeame. Ema fia be ƒã ŋutɔ. Gake amesiwo ŋu dɔléle sia le la ate ŋu axɔ anaphylaxis ( nulɔdɔ sesẽ aɖe si nu sẽ, kpata, si ate ŋu ade woƒe agbe afɔku me). Ƒã ŋutɔ la, ``Systemic Mastocytosis'' hã ateŋu atrɔ azu kansa. Ðɔktawo mate ŋu ada dɔléle sia keŋkeŋ o. Gake atikewɔmɔnu aɖewo li siwo ate ŋu akpe ɖe ŋuwò nàɖu wò dzesiwo dzi ahanɔ agbe si sɔ.

Nukae nye `(Systemic Mastocytosis)` ƒe ƒomevi veviwo?

``Systemic Mastocytosis`` ƒomevi vevi ade ye li. Wokpɔa ŋusẽ ɖe mía dzi le mɔ vovovowo nu. Le goawo katã me la, zi alesi mast cells siwo mesɔ o le wò ŋutilã me la, zi nenemae wòate ŋu adze kuxiwo gɔmee. Mina míalé ŋku ɖe nusiwo ƒomevi mawo nye ŋu: 1. Ƒomevi si tsina blewu (``Indolent systemic mastocytosis``):

• Esiae nye ƒomevi si bɔ wu. Abe ati si tsina blewu ene la, ate ŋu axɔ ƒe geɖe hafi dzesiwo nadze. Mast cell siwo mesɔ o ƒe xexlẽme dzina ɖe edzi le ɣeyiɣi aɖe megbe. Esia ate ŋu ahe dzesiwo vɛ le ŋutigbalẽ, aklã , dɔgbo , kple dɔgbo ƒe dɔwɔwɔ (i.e., dɔgbowo) me.

2. Ƒomevi si de ŋgɔ vie wu (`Systemic smoldering mastocytosis`):

• Le lãmenugbagbevi sia ƒomevi me la, mast cell siwo mesɔ o ƒoa ƒu ɖe aklã kple aklã me le agbɔsɔsɔ gã me. Le ɣeyiɣi aɖe megbe la, esia ate ŋu ana aklã alo dɔgbo navuvu. Bu eŋu abe nusi dzɔna ne aƒe sue aɖe me yɔ fũu akpa ene.

3. Ƒomevi si va kple ʋudɔ bubuwo (`Systemic mastocytosis with associated hematologic neoplasm`):

• Ame siwo ŋu dɔléle sia ƒomevi le ate ŋu axɔ dɔléle bubu siwo do ƒome kple ʋu, abe myeloproliferative neoplasms kple myelodysplastic syndrome . Esia ƒomevi dzea amesiwo ade ɖeka le atɔ̃ ɖesiaɖe me si ŋu Systemic Mastocytosis le dzi.

4. Ƒomevi sesẽ (`Aggressive systemic mastocytosis`):

• Esia gavloe vie elabena ate ŋu agblẽ nu le ƒumeŋuɖui (afisi wowɔa ʋumenugbagbeviwo le) kple ƒuwo ŋu. Mast cell siwo mesɔ o ate ŋu atsi le ƒuawo me, si ana woagbɔdzɔ eye wòana woagbã bɔbɔe.

5. Ƒomevi si trɔna zua ʋumenugbagbeviɣidɔ (`Mast cell leukemia`):

• Ƒã ŋutɔ hafi Systemic Mastocytosis ate ŋu ava zu nɔnɔme si woyɔna be Mast Cell Leukemia . Esia nye Acute Myeloid Leukemia (AML) ƒomevi aɖe .

6. Dɔdzẽ ƒomevi (`Mast cell sarcoma`):

• Esiae nye ne dɔdzẽ (tumor) siwo wowɔ tso mast cells siwo mesɔ o me gblẽa nu le ŋutilãa ƒe lãkusi vovovowo ŋu. Ame ʋɛ aɖewo ŋutɔ siwo ŋu ``Systemic Mastocytosis`` le la xɔa nɔnɔme sia si woyɔna be ``Mast cell sarcoma``.

Nukae nye dɔléle sia (Systemic Mastocytosis) ƒe dzesiwo?

Dzesiawo ate ŋu ato vovo tso ame ɖeka gbɔ yi bubu gbɔ. Enɔ te ɖe afisi mast cell siwo mesɔ o la le wu le ŋutilãa me dzi. Le kpɔɖeŋu me, ne mast cells sɔ gbɔ ɖe wò dɔgbo me la, àte ŋu axɔ abi kple dɔgbo ƒe vevesese. Azɔ hã, ne mast cells ƒo ƒu ɖe wò ƒumeŋuɖui me la, ate ŋu akpɔ ŋusẽ ɖe ʋumenugbagbeviwo ƒe wɔwɔ dzi. Dzesi siwo bɔ dometɔ aɖewoe nye esi:

• Ʋusɔgbɔdɔ ( ʋu ƒe veve ) .
• Ƒu ƒe vevesese
• Ʋu si le dodom fũu akpa (e.g., ʋu gbogbo aɖe si dona tso abi sue aɖe me) .
• Ðeɖiteameŋu ƒe seselelãme ɣesiaɣi (` Ðeɖiteameŋu` ) .
• Ŋutilã si biã
• Dzi ƒe tsotso
• Hives (aɖuɖɔtoe, ʋuʋudedi siwo kɔ ɖe ŋutigbalẽ ŋu) .
• Ŋutilã si le ʋuʋum
• Seselelãme ƒe tɔtrɔ alo blanuiléleblanuiléle `) .
• Teƒe doviviti siwo le ʋuʋum le ŋutigbalẽa ŋu (`Urticaria pigmentosa`) .

Ɣeaɖewoɣi la, amesiwo ŋu Systemic Mastocytosis le ate ŋu akpɔ dzesi siawo dometɔ geɖe zi ɖeka. Ðɔktawo yɔa esia be "mastocytosis attack" alo "flare."

Vevietɔ: Menye amesiame si ŋu dzesi siawo le si ŋu `(Systemic Mastocytosis) le o.` Gake ne dzesi siawo dometɔ geɖe le ŋuwò zi ɖeka, eye woyi edzi la, ele be nàyi ɖɔkta gbɔ godoo be wòaɖo aɖaŋu na wò.

Nukatae `(Systemic Mastocytosis)` sia dzɔna?

Numekukuwo ɖee fia be amesiwo ŋu `(Systemic Mastocytosis)` le ƒe 80% lɔƒo ƒe `KIT domenyiŋusẽfianu ƒe tɔtrɔ` le wo si. `KIT` domenyiŋusẽfianu sia kpena ɖe lãmenugbagbevi ƒomevi aɖewo ŋu le míaƒe ŋutilã me, vevietɔ ʋumenugbagbeviwo kple mast cells, be woatsi. Domenyiŋusẽfianu ƒe tɔtrɔ sia dzɔna ne míefɔ fu le vidzidɔa me vɔ. Esia fia be menye domenyinu o.

Nukae nye nusiwo nana dɔlélea nu sẽna ɖe edzi alo doa dziku (`Triggers`)?

Nu geɖewo ateŋu ahe ``Systemic Mastocytosis.`` ƒe dzesiwo vɛ.`` Menye amesiamee nu ɖeka ma ke gblẽa nu le o. Gake nu ʋɛ aɖewo li siwo bɔ:

• Ahasesẽ
• Nuɖuɖu siwo me wodea nu ʋeʋĩiwoe
• Atike aɖewo: Le kpɔɖeŋu me , NSAID ( vevesesenutsitike), lãmekawo ƒe ɖiɖiɖeme, kple alɔ̃dɔdɔ.
• Kamedede kple kamedede ( na ame aɖewo ko) .
• Nudzodzoewo ƒe ɖuɖu (vevietɔ anyidiwo, ʋetsuviwo, kple anyiwo) .
• Ŋutilã alo seselelãme ƒe nuteɖeamedzi
• Wò ŋutigbalẽ tutu alo eƒe ʋuʋu
• Dzoxɔxɔ ƒe tɔtrɔ kpata (e.g., titri kpata ɖe tsi fafɛ me) .

Ne dɔléle sia le ŋuwò la, ele vevie ŋutɔ be nàbia wò ɖɔkta be nukae nye wò gbɔdzɔgbɔdzɔwo kple nusiwo wòle be nàƒo asa na hã.

Aleke nèwɔna dea dzesi dɔléle sia pɛpɛpɛ? (Dɔléle ƒe dzesi) .

Ne èyi ɖɔkta gbɔ la, nu gbãtɔ si woawɔe nye be woado wò ŋutilã kpɔ. Woabia nya tso wò dzesiwo kple dɔléle ɖe sia ɖe si nèdze va yi ŋu. Emegbe, woate ŋu awɔ dodokpɔ siwo gbɔna la dometɔ ɖeka alo esi wu nenema atsɔ anya ne Systemic Mastocytosis le ŋuwò:

• Ʋudodokpɔ: Esia léa ŋku ɖe tryptase ƒe agbɔsɔsɔ si le wò ʋu me ŋu. Tryptase nye enzyme tɔxɛ aɖe si mast cells ɖena ɖe go ne wowɔ nu ɖe ​​dutanuwo ŋu.
• Ƒuwo me toto: Kpɔe ɖa be ƒuwo gblẽ hã.
• Ƒumeŋuɖui ƒe lãmenugbagbeviwo ɖeɖe: Esia bia be nàɖe wò ƒumeŋuɖui ƒe kpɔɖeŋu sue aɖe ahadoe kpɔ be mast cells siwo mesɔ o le eme hã, eye wosɔ gbɔ.
• Domenyiŋusẽfianu dodokpɔwo: Kpɔe ɖa be tɔtrɔ aɖe le `KIT` domenyiŋusẽfianu si míeyɔ va yi me hã.

Aleke wowɔa atike nɛ?

Ðɔktawo daa Systemic Mastocytosis to dzesiwo kple kuxiwo dzi ɖuɖu me. Le kpɔɖeŋu me, ne dɔgbo me acid sɔ gbɔ ɖe lãme na wò la, woate ŋu aŋlɔ H2 blockers (si nye antacid ƒomevi aɖe) na wò. Ne ʋusɔgbɔdɔ tso dɔlélea gbɔ la, woadae. Atikewɔwɔ vevi aɖewoe nye esi:

• Antihistamines: Ðe ŋutigbalẽ ƒe dzesiwo abe ʋuʋudedi kple dzodzro ene dzi kpɔtɔna.
• Corticosteroids: Eɖea abi kple abi si le ŋutilãa me dzi kpɔtɔna.
• Bisphosphonates: Wokpena ɖe ƒuwo ŋu wodoa ŋusẽ wo ne wogbɔdzɔ.
• Atikewɔwɔ si woɖo taɖodzinu na: Esia bia be woaɖo taɖodzinu na protein aɖe koŋ si le mast cells siwo mesɔ o me.
• Atikewɔwɔ: Ne Systemic Mastocytosis trɔ zu kansa ko hafi wozãa esia.
• Aɖuɖɔtoe ɖeɖe ɖa: Ne dɔgboa vuvu ŋutɔ la, woate ŋu awɔ dɔ nɛ atsɔ aɖee ɖa.
• Ƒumeŋuɖui dodo: Wowɔa esia na amesiwo ŋu dɔléle sesẽ ŋutɔ le, alo amesiwo le eƒe akpa mamlɛawo me.

Ðo ŋku edzi be: Ne Systemic Mastocytosis le ŋuwò la, ele be nàtsɔ EpiPen . EpiPen nye atike si woate ŋu azã enumake ne nulɔdɔ sesẽ aɖe le ame ŋu, abe Anaphylaxis si míeyɔ va yi ene.

Ðe womate ŋu axe mɔ ɖe ``Systemic Mastocytosis'' sia nu oa?

Abe alesi míegblɔe va yi ene la, ``Systemic Mastocytosis`` tso domenyiŋusẽfianu ƒe tɔtrɔ gbɔ. Eyata manya wɔ be woaxe mɔ nɛ be wòagatsi o. Ke hã, àte ŋu aɖu dɔlélea dzi ne èƒo asa na ``nu siwo hea dɔlélea vɛ`` siwo dzia dzesiwo ɖe edzi.

Etsɔme ka ƒomevie le amesi ŋu `(Systemic Mastocytosis)` le?

Esia nɔ te ɖe `(Systemic Mastocytosis)` ƒomevi si le asiwò dzi ŋutɔŋutɔ. Zi geɖe la, amesiwo ƒe ƒomevi si tsina blewu (`Indolent systemic mastocytosis`) le ate ŋu aɖu dɔlélea dzi nyuie ne wodae. Woate ŋu anɔ agbe si sɔ. Gake amesiwo ŋu dɔléle sesẽ bubuawo le ƒe agbenɔƒe ate ŋu anɔ kpuie vie. Ke hã, woateŋu ada `(Systemic Mastocytosis)` keŋkeŋ to atikewɔwɔ si woyɔna be ``Bone marrow transplant`` me. Gake amesiwo le dɔlélea ƒe sesẽme wu me koe ɖɔktawo naa atike ma.

Aleke malé be na ɖokuinye esime mele agbe kple `(Systemic Mastocytosis)`?

Nusiwo hea wò dzesiwo vɛ nyanya kple wo ƒoƒo asa na nye nu vevitɔ kekeake si nàte ŋu awɔ ne èle agbe kple Systemic Mastocytosis. Ne nɔnɔme sia le ŋuwò la, ke nya nu tso nu siawo ŋu:

• Tsɔ EpiPen ɖe asi ɣesiaɣi. Da nulɔdɔ sesẽ aɖe kaba.
• Nya nusiwo tututu wòʋãa wò eye nàƒo asa na wo alesi nàte ŋui.
• Kpɔ nuteɖeamedzi dzi. Srɔ̃ ŋugbledede alo susuŋudɔwɔwɔ ƒe mɔnu bubuwo.
• Do asigɛ si naa atikewɔwɔ ŋuti nuxlɔ̃ame si dzi woŋlɔ atike siwo màte ŋu ano o ɖo. Esia le vevie ŋutɔ elabena ekpena ɖe ɖɔktawo ŋu wonya wò nɔnɔmea ne mète ŋu ƒoa nu le nɔnɔme kpata aɖe me o.

Ɣekaɣie wòle be makpɔ ɖɔkta?

Ne Systemic Mastocytosis le ŋuwò la, ahiã be nàyi ɖɔkta gbɔ be woado wò kpɔ edziedzi. Azɔ hã, kpɔ egbɔ be yegblɔe na wò ɖɔkta ne dzesi yeye aɖewo dze le ŋuwò, alo ne edze abe wò dzesi aɖewo le sesẽm ɖe edzi ene.

Nya kawoe wòle be mabia ɖɔktaa?

Esi wònye be Systemic Mastocytosis nye dɔléle si mebɔ o ta la, nyabiase geɖe ate ŋu anɔ asiwò. Nya aɖewo siwo wòle be nàbia wò ɖɔktae nye esi:

• ``Systemic Mastocytosis'' ƒomevi kae le ŋunye?
• Dzesi bubu kawoe ate ŋu afia be nye nɔnɔmea le sesẽm ɖe edzi?
• Atikewɔwɔ kawoe li na nye ƒomevi?
• Ðe atikewɔwɔa aɖe nye dzesiawo ɖa keŋkeŋa?
• Ðe dɔdamɔnu dodokpɔ aɖewo li siwo me mate ŋu akpɔ gome lea?

Ne wokpɔ be Systemic Mastocytosis le ame ŋu la, enye nuteƒekpɔkpɔ si trɔa ame ƒe agbenɔnɔ. Ele be nàyi ɖɔkta gbɔ edziedzi, ado ʋu kpɔ, eye nàsrɔ̃ alesi nàde dzesi nusiwo aʋã wò ahaƒo asa na wo. Agbenɔnɔ kple nɔnɔme sia nye tɔtrɔ gã aɖe. Eyata, lé ƒomedodo nyui me ɖe asi kple wò ɖɔkta. Wole afima be yewoakpe ɖe ŋuwò. Woana nyatakakawo kple kpekpeɖeŋunaha siwo ate ŋu akpe ɖe ŋuwò la wò. Esiawo ana ŋusẽ wò be nàwɔ nyametsotsowo le wò lãmesẽ ŋu.

Mlɔeba la, nusiwo dzi wòle be woaɖo ŋkui (Take-Home Message) .

Enyo, eyata míeƒo nu geɖe tso `(Systemic Mastocytosis)` ŋu, alo? Ðo ŋku edzi be togbɔ be dɔléle si mebɔ o nye esia hã la, ele vevie ŋutɔ be nànɔ ŋudzɔ ɖe eŋu.

Nu vevitɔe nye be nàyi ɖɔkta gbɔ ne dzesi siawo le ŋuwò. Ne wokpɔ dɔlélea kaba la, woate ŋu adze edada gɔme eye woate ŋu aɖu dzesiawo dzi.

Menye wò ɖeka koe o. Wò ɖɔktawo, wò ƒometɔwo, kple xɔlɔ̃wo le afima be yewoakpe ɖe ŋuwò. Ne ènya nyatakaka siwo sɔ eye nèwɔ ɖe wò ɖɔkta ƒe aɖaŋuɖoɖo dzi la, àte ŋu anɔ agbe nyuie kple nɔnɔme sia. Nɔ anyi kpoo eye nàbu nu nyuiwo ŋu!