So wotaa nya akisikuru a ɛwɔ nsensanee kɔkɔɔ ne ɛyaw wɔ wo nipadua nyinaa so? Eyi betumi ayɛ Mastocytosis!

So wotaa nya akisikuru kɔkɔɔ, ɛyɛ wo yaw, na ɛtɔ mmere bi a emu yɛ den wɔ wo nipadua so? Anaasɛ wo ho yɛ wo hyew kɛse wɔ ade ketewaa bi mpo ho nna pii? Ebia wubesusuw sɛ, "Mewɔ allergy foforo." Nanso, ɛtɔ mmere bi a, wɔ saa sɛnkyerɛnne ahorow yi akyi no, ebia tebea bi a ɛntaa nsi wɔ hɔ a yɛntee ho asɛm. Tebea a ɛte saa no biako ne `(Mastocytosis)`. Ɛwom sɛ ebia edin no bɛyɛ te sɛ nea ɛyɛ nwonwa kakra de, nanso momma yɛnka ho asɛm tiawa.

Dɛn ne Mastocytosis? Sɛ yɛbɛka no tiawa a...

Fa no sɛ, nkwammoaa bi wɔ yɛn nipadua mu a ɛte sɛ awɛmfo nketewa. Yɛfrɛ wɔn ``mast cells.`` Saa ``mast cells`` yi yɛ yɛn ``immune system no fã.`` Wɔte sɛ yɛn man no ahobammɔ asraafo. Sɛ atamfo te sɛ mmoawa, mmoawa, ne nneɛma a ɛma obi ho yɛ no hyew hyɛn nipadua no mu a, saa ``mast cells`` yi ba anim na wɔko.

Sɛ saa `(mast cells)` yi yɛ wɔn ade wɔ atamfo ho a, woyi nnuru bi a wɔfrɛ no `(histamine) fi adi.` Saa `(histamine)` yi na ɛde akisikuru ho sɛnkyerɛnne ba yɛn nipadua mu (sɛ nhwɛso no, ɛyaw, honam ani kɔkɔɔ, ɛho ahonhon). Saa `(mast cells)` yi nso boa ma wɔyɛ acid wɔ yafunu no mu.

Mpɛn pii no, saa `(mast cells)` yi yɛ wɔn adwuma na wodi atamfo no so na afei wɔyɛ komm bio. Nanso, nea ɛkɔ so wɔ obi a ɔwɔ `(Mastocytosis)` nipadua mu ne sɛ saa `(mast cells)` yi kɔ soro wɔ ɔkwan a ɛnteɛ so na wontumi nni so. Sɛ wɔdɔɔso saa a, saa nkwammoaa yi gyae `(histamine) pii dodo.` Ɛno ne bere a wo nya allergy kɛse wɔ ade ketewaa bi mpo ho, anaasɛ sɛ wo nya allergy a, ɛyɛ bɔne sen onipa a ɔte ase, na ɛtra hɔ kyɛ.

Nea ɛho hia sen biara no, obiara a ɔwɔ Mastocytosis no wɔ asiane kɛse mu sɛ obenya anaphylaxis , akisikuru a ɛba mpofirim, emu yɛ den, na ebetumi de ne nkwa ato asiane mu . Enti, ɛho hia sɛ wohu eyi.

Ɛwom sɛ nnuruyɛfo ntumi nsa Mastocytosis koraa de, nanso wobetumi adi sɛnkyerɛnne ahorow no so na wɔasa nsɛnnennen biara a ebetumi aba.

Dɛn ne Mastocytosis ahorow atitiriw?

Saa yare yi ahorow atitiriw abien na ɛwɔ hɔ:

1. Cutaneous Mastocytosis: Eyi taa ba nkokoaa ne mmofra nkumaa so . Nanso, ebetumi aba mpanyimfo nso. Nea ɛkɔ so wɔ ha ne sɛ ``mast cells`` a ɛnyɛ ne kwan so boaboa ano wɔ honam ani, na ɛde akisikuru ne nsensanee a ɛyɛ kɔkɔɔ, kɔkɔɔ tuntum, bruu, anaa kɔla afrafra ba. Ɛte sɛ nea abofra ketewa bi nya nsensanee kɔkɔɔ pii mpofirim wɔ wɔn nipadua so na wɔtwetwe wɔn a wonnyae.

2. Systemic Mastocytosis: Saa ɔkwan yi na wɔtaa hu wɔ mpanyimfo mu.Ɛha yi, ``mast cells`` a ɛnyɛ ne kwan so boaboa ano wɔ mu akwaa te sɛ nnompe mu ntini, nnompe, yɛn aduan mu nhyehyɛe, ne ahurututu mu. Fa no sɛ, sɛ saa nkwammoaa yi boaboa ano boro so wɔ nnompe mu a, ebetumi aka yɛn mogya mu nkwammoaa a wɔhyehyɛ no. Afei nso, sɛ saa nkwammoaa yi dɔɔso wɔ yafunu mu a, ebetumi ama yafunu mu asikre adɔɔso dodo na ama yafunu mu akuru aba.

So eyi yɛ yare a abu so?

Dabi, nokwarem no, eyi yɛ tebea a ɛntaa nsi koraa. Sɛnea nhwehwɛmu ahorow kyerɛ no, wɔka sɛ nnipa mpem du (10,000) biara mu biako anaa mpem aduonu (20,000) biara mu biako pɛ na yare yi hu amane. Enti, ɛyɛ ade a ɛfata sɛ ɛnyɛ obiara na onim ho asɛm.

Dɛn ne Mastocytosis ho sɛnkyerɛnne ahorow a ebetumi aba?

Bere a saa ``mast cells`` yi kɔ soro wɔ ɔkwan a ɛnteɛ so no, sɛnkyerɛnne ahorow betumi aba. Bere a saa sɛnkyerɛnne ahorow yi betumi ayɛ nea emu nyɛ den koraa wɔ nnipa binom fam no, ebetumi ayɛ den ama afoforo.

• Nnompe mu yaw: Wote nka te sɛ nea wo nnompe reyɛ wo yaw.
• Atiridiinini (yafunu mu yaw): Ɛsɛ sɛ wɔtaa kɔ aguaree.
• Totɔ piti (te sɛ adwene a ɛyera): Mpofirim ara, aniwa no dan bruu na ɛte sɛ nea w’adwene atu afra.
• Adwennwene ne ehu tebea a ɛkɔ so daa: Dadwen a wɔte nka a ntease biara nnim.
• Flushing: Wora a ɛyɛ kɔkɔɔ mpofirim wɔ nipadua no nyinaa so.
• Akisikuru anaa nsensanee a ɛyɛ yaw wɔ honam ani: Sɛnea yɛadi kan aka ho asɛm no.
• Tiyɛ: Tiyɛ a ɛtaa yɛ no yaw.
• Adwene a ɛyɛ basaa a mogya mmoroso a ɛba fam de ba: Adwene a ɛyɛ basaa bere a obi gyina hɔ fi gyinabea no.
• Nkwaa mu yaw: Nkwaa mu yaw.
• Nsonsonoe kɔkɔɔ anaa bruu a ɛwɔ honam ani: Eyinom betumi ayɛ wo yaw anaasɛ ɛrenyɛ wo yaw.

Mfe bɛn na Mastocytosis fi ase?

Ɛsono sɛnea yare no te. Sɛ yɛbɛka no ɔkwan biara so a:

• Cutaneous Mastocytosis: Eyi betumi aka nkokoaa a wɔawo wɔn foforo mpo.
• Systemic Mastocytosis: Ɛtaa ka mpanyimfo a wɔadi boro mfe 50.

Dɛn nti na ɛte saa?

`(Mastocytosis)` nnyɛ biribi a wɔde awo yɛn. Ɛnam nkwaadɔm mu nsakrae a ɛba awosu mu nkwaadɔm a wɔfrɛ no `(KIT awosu mu nkwaadɔm)` mu. Saa `(KIT genes)` yi yɛ awosu mu nkwaadɔm a ɛboa nkwammoaa ahorow bi, te sɛ yɛn mogya mu nkwammoaa, `(mast cells)`, nyin. Wɔ `(Mastocytosis) mu no, saa `(KIT awosu mu nkwaadɔm)` a wɔayɛ no foforo yi de sɛnkyerɛnne a ɛnteɛ ma `(mast cells)` sɛ "ɛdɔɔso dodo na enyin a wontumi nni so."

Dɛn ne nneɛma a ɛkanyan mastocytosis?

Eyi betumi ayɛ soronko wɔ onipa biara ho. Ɛnyɛ obiara na ɔwɔ nneɛma a ɛkanyan no koro. Nanso, nneɛma bi wɔ hɔ a mpɛn pii no ebetumi ama yare no ho sɛnkyerɛnne akɔ soro:

• Wo honam ani a wobɛpetepete anaa wobɛtwetwe: Eyi nso betumi ato nnipa binom denam ntade a ɛyɛ den a wɔhyɛ ara kwa so.
• Apɔw-mu-teɛteɛ ne apɔw-mu-teɛteɛ a ɛtra so.
• Nkoekoemmoa a wɔwe wɔn anaa wɔka wɔn:Titiriw mmoa te sɛ ntɛtea, ntɛtea, ne ntɛtea.
• Nsã a wɔnom.
• Nnuan bi: Titiriw nnuan a ɛyɛ nnuhwam a wɔde ahyɛ mu ma.
• Nnuru binom: Sɛ nhwɛso no, nnuru a wɔde kum ɛyaw a wɔfrɛ no ``NSAIDs`` (Nonsteroidal anti-inflammatory drugs), nnuru a ɛma ntini mu dwo, ne nnuru a ɛma obi dwensɔ ``(anesthesia)``.
• Ɔhyew a ɛsakra mpofirim: Te sɛ nea wuhuruw kɔ nsu a ɛyɛ nwini paa mu.
• Nipadua anaa adwene mu adwennwen: Eyi taa nya nkɛntɛnso kɛse.

Dɛn ne nsɛnnennen a ebetumi afi Mastocytosis mu aba?

Saa yare yi betumi de akwahosan ho haw afoforo aba, a nea ɛka ho ne:

• Nnompe mu yare: Eyi ma nnompe yɛ mmerɛw na ɛnyɛ den sɛ ebebubu.
• Mmɛn ho haw: Tebea a ɛma mmerɛbo no hohoro na ɛnyɛ adwuma yiye.
• Cytopenia: Nipadua no ntumi nnya mogya mu nkwammoaa a ɛdɔɔso.
• Nyarewa a wɔtaa nya.

Ɔkwan bɛn so na wohu Mastocytosis?

Wo duruyɛfo bebisa wo yare no ho sɛnkyerɛnne na wahwɛ wo honam ani. Nea ɛka ho no, wobetumi ayɛ sɔhwɛ ahorow te sɛ:

• Mogya mu nhwehwɛmu: Hwɛ sɛnea enzyme bi a wɔfrɛ no ``tryptase`` dodow wɔ wo mogya mu. Saa ``tryptase`` yi yɛ biribi a ``mast cells`` gyae bere a wɔyɛ atamfo ho biribi.
• Bone scan: Sɛnea ɛbɛyɛ a wobɛhwɛ sɛ nnompe asɛe anaa.
• Bone marrow biopsy: Wɔfa nnompe mu ntini bi na wɔhwɛ sɛ mast cells a ɛnteɛ anaa.
• Awosu mu nhwehwɛmu: Sɛ wowɔ Systemic Mastocytosis a, wobetumi asɔ wo ahwɛ sɛ ebia nkwaadɔm mu nsakrae bi aba KIT awosu mu nkwaadɔm mu anaa.

Dɛn ne ayaresa ahorow a wɔde sa Mastocytosis?

Ɛsono ayaresa a wobɛpaw a egyina yare no ko ne wo sɛnkyerɛnne ahorow so. Nnuruyɛfo taa de nnuru ne ayaresa afoforo di dwuma de ma yare no ho sɛnkyerɛnne no fi hɔ.

Nnuru ahorow a wɔde yɛ Mastocytosis:

• Antacids ma yafunu mu haw (Antacids - H2 blockers): Ma nneɛma te sɛ yafunu mu ɔfe ne yafunu mu yare.
• Nnuru a ɛko tia histamine: Ɛtew honam ani yare a ɛma obi ho yɛ no hyew te sɛ ɛyaw ne kɔkɔɔ so.
• Bisphosphonates a wɔde sa nnompe mu yare: Ɛhyɛ nnompe den.
• Corticosteroids: Ɛtew ɔfe so.
• Monoclonal antibodies: titiriw nnuru te sɛ omalizumab (Xolair®).
• Aduru a wɔde sa ɛyaw .

Ayaresa afoforo:

• Ultraviolet (UV) hann ayaresa: Tew nsensanee tuntum a ɛwɔ honam ani no so.
• Ayaresa a wɔde wɔn ani asi so .
• Epinephrine a wɔde sa anaphylaxis:Ayaresa a egye ntɛmpɛ ma obi a ne ho yɛ no yaw kɛse.
• Nnuru a wɔde sa yare: Eyi ho yɛ na koraa na sɛ Mastocytosis dan kokoram nkutoo a, wɔde ma.
• Allogeneic stem cell (nnompe mu ntini) a wɔde si ananmu: Wosusuw eyi nso ho wɔ mastocytosis a emu yɛ den yiye na ɛyɛ den mu. Nnipa dodow no ara nhia eyi.

So nsɛnnennen anaa nsunsuanso bɔne bi wɔ ayaresa no mu?

Yiw, sɛnea ɛte wɔ ayaresa biara ho no, nsunsuanso bɔne bi wɔ hɔ a ebetumi aba wɔ Mastocytosis ayaresa mu. Ebia ebinom yɛ nketewa, na ebinom nso betumi ayɛ aniberesɛm kɛse. Sɛ wowɔ Mastocytosis a, wo duruyɛfo bɛkyerɛkyerɛ ayaresa ahorow a woreyɛ ne nea ebetumi afi mu aba no mu.

So wobetumi asa Mastocytosis koraa?

Sɛ yɛbɛka no ɔkwan biara so a, wobu Mastocytosis sɛ yare a wontumi nsa koraa, nanso wobetumi adi ho dwuma denam sɛnkyerɛnne ahorow a wobedi so ne nsɛnnennen a wɔbɛsa so .

Ayaresa biako pɛ a ebetumi asa eyi ne ``allogeneic stem cell (bone marrow) transplantation'' a yɛadi kan aka ho asɛm no. Nanso, wɔkamfo ɛno mpo kyerɛ ayarefo a wɔwɔ tebea horow a emu yɛ den na emu yɛ den sen biara nkutoo.

So Mastocytosis mu yɛ den bere a bere kɔ so no?

Sɛ wɔansa a, bere kɔ so no, sɛnkyerɛnne ahorow no betumi asɛe kɛse, titiriw wɔ obi a ɔwɔ systemic mastocytosis , bere a mast nkwammoaa a ɛnteɛ no kɔ so ka nnompe mu ntini ne emu akwaa ahorow no.

So wobetumi asiw Mastocytosis ano?

Nea ɛyɛ awerɛhow no, dabi. Esiane sɛ nkwaadɔm mu nsakrae a ɛba KIT awosu mu nkwaadɔm mu na ɛde ba nti, ɔkwan biara nni hɔ a wɔbɛfa so asiw ano. Nanso, sɛ wowɔ Mastocytosis a, wubetumi adi wo sɛnkyerɛnne ahorow no so denam nea ɛkanyan wo ho a ɛyɛ wo yaw ankasa a wubehu na woakwati sɛnea wubetumi biara no so.

Sɛ nhwɛso no, sɛ wowɔ biribi a ɛkanyan nnuru bi a, ɛyɛ papa sɛ wobɛhyɛ aduruyɛ ho kɔkɔbɔ abɔso a wɔakyerɛw so. Eyi bɛma afoforo ahu nnuru a ɛnsɛ sɛ wonom sɛ biribi si ntɛm a.

Sɛ wowɔ Systemic Mastocytosis a, ɛho hia sɛ wode syringe a wode twitwiw wo ho a epinephrine wom bɛkɔ bere nyinaa. Wobetumi de eyi asa akisikuru a emu yɛ den a ɛde nkwa to asiane mu te sɛ anaphylactic shock.

Mɛyɛ dɛn ahwɛ me ho? (Mastocytosis) Bere a ɔne...

Esiane sɛ Mastocytosis yɛ yare a wontumi nsa koraa nti, ebia ɛsɛ sɛ wo ne no tra ase kakra. Ebetumi ayɛ den. Nanso, nneɛma bi a ebetumi aboa wo ni:

• Hu nea ɛkanyan wo yare no ho sɛnkyerɛnne no ankasa.Ɛsono nea ɛkanyan obiara. Ebia wobɛpɛ sɛ wokyerɛw da biara da na wokyerɛw bere a wo yare no ho sɛnkyerɛnne no mu yɛ den, nea wudii ne nea wonom, ne nea woyɛe saa bere no.
• Adwennwen betumi ama saa sɛnkyerɛnne ahorow yi asɛe kɛse. Sɛ wuhia mmoa a wode bedi adwennwen ho dwuma a, bisa wo duruyɛfo nhyehyɛe ahorow a wɔde siw adwennwen ano ho asɛm. Ebia nneɛma te sɛ adwennwen ne yoga bɛboa.
• Hyɛ abɔso a wɔde bɔ kɔkɔ wɔ aduruyɛ mu. Ɛsɛ sɛ ɛkyerɛw w’ayaresa tebea ne nnuru biara a ɛnsɛ sɛ wonom.
• Fa epinephrine auto-injector needle ka wo ho bere nyinaa. Ebetumi agye nkwa sɛ ɛba sɛ obi ho yɛ no yaw kɛse a.

Bere bɛn na ɛsɛ sɛ mekɔ me duruyɛfo nkyɛn?

Wo ne wo duruyɛfo bɛkɔ akɔhwɛ wo daa . Wɔbɛhwɛ wo sɛnkyerɛnne ahorow no so na sɛ ɛho hia a, wɔbɛsesa w’ayaresa no. Sɛ wunya sɛnkyerɛnne foforo, anaasɛ wo sɛnkyerɛnne no mu yɛ den a, ka kyerɛ wo duruyɛfo ntɛm ara.

Bere bɛn na ɛsɛ sɛ mekɔ Emergency Room?

Sɛ wowɔ akisikuru a emu yɛ den yiye a wuntumi mfa nnuru nni dwuma a, kɔ baabi a wɔhwɛ wɔn a wɔwɔ yare a egye ntɛmpɛ ntɛm ara. Ɛne sɛ, sɛ wuhu sɛnkyerɛnne ahorow te sɛ ɛyɛ den sɛ wobɛhome, wo menewa mu ayɛ wo den, wo ho yɛ wo yaw kɛse, anaasɛ wo ho atɔ wo.

Nsɛm bɛn na ɛsɛ sɛ mibisa me duruyɛfo?

Sɛ wowɔ Mastocytosis a, ɛyɛ papa sɛ wubebisa wo duruyɛfo nsɛm te sɛ eyinom:

• Mastocytosis bɛn na mewɔ?
• Ayaresa bɛn na ɛwɔ hɔ ma ɔkwan a mewɔ no?
• So ayaresa no beyi me yare no ho sɛnkyerɛnne no afi hɔ koraa?
• Nsɛnkyerɛnne foforo bɛn na ebetumi akyerɛ sɛ me tebea no reyɛ kɛse?
• So ɛsɛ sɛ mefa ayaresa daa?
• Sɛ mewɔ ɔkwan a emu yɛ den kɛse a, dɛn ne me nkɔmhyɛ?
• So nnuruyɛ mu sɔhwɛ ahorow bi wɔ hɔ a metumi de me ho ahyɛ mu?

Awiei koraa no, nea ɛsɛ sɛ wokae (Take-Home Message) .

Mastocytosis yɛ tebea a ɛntaa nsi a ɛma mast nkwammoaa nyin wɔ nipadua no mu wɔ ɔkwan a ɛnteɛ so a wontumi nni so. Ebetumi aka honam ani akwaa ne emu akwaa ahorow. Nnipa dodow no ara betumi adi wɔn sɛnkyerɛnne ahorow ho dwuma denam nneɛma a ɛkanyan no a wobehu na wɔakwati ne nnuru a wɔnom so. Ɛho hia nso sɛ wode epinephrine a wɔde twitwiw nipadua no si wo nsam de sa akisikuru a emu yɛ den.

Ɛwom sɛ eyi yɛ yare a wontumi nsa koraa de, nanso sɛ wunya aduruyɛ mu afotu ne ayaresa a ɛfata a, wubetumi abɔ mmɔden sɛ wubedi yare no ho sɛnkyerɛnne no so na woabɔ bra sɛnea ɛsɛ. Sɛ wowɔ nsɛmmisa foforo bi fa eyi ho a, ntwentwɛn wo nan ase sɛ wo ne wo duruyɛfo bɛkasa. Wɔbɛboa wo.

` Mastocytosis, mast cells, histamine, allergy, honam ani nyarewa, nipadua mu ahoɔden a ɛko tia nyarewa, anaphylaxis