So wotaa nya nipadua mu yaw a ɛyɛ nwonwa? Momma yensua pii fa saa (Systemic Mastocytosis) yi ho!

So ɛtɔ mmere bi a, wo honam ani yɛ kɔkɔɔ na ɛyɛ wo yaw? Anaasɛ wo yafunu yɛ wo yaw na wo ti yɛ wo yaw a ɛba na ɛkɔ kɛkɛ? Sɛ saa nneɛma yi si mpɛn pii a, ebetumi ayɛ tebea bi a ɛntaa nsi a wɔfrɛ no ``Systemic Mastocytosis'' a yɛbɛka ho asɛm nnɛ. Mma ɛnhaw wo, eyi nyɛ yare a ɛka nnipa pii. Nanso ɛho hia sɛ wohu eyi.

Enti, dɛn ne saa ``Systemic Mastocytosis'' yi?

Sɛ yɛbɛka no tiawa a, `(Systemic Mastocytosis)` yɛ yareɛ a ɛntaa nsi a ɛfa yɛn mogya ho. Nea ɛkɔ so wɔ eyi mu ne sɛ nkwammoaa soronko bi a wɔfrɛ no `(Mast cells)` a ɛwɔ yɛn nipadua mu no nyin wɔ ɔkwan a ɛnteɛ so. Susuw ho hwɛ, saa mast cells yi te sɛ asraafo nkumaa wɔ yɛn nipadua mu. Wɔn na sɛ́ yɛn nipadua mu ahoɔden a ɛko tia nyarewa no fã no, wohu ahɔho a wɔtow hyɛ wɔn so (te sɛ mmoawa, nneɛma a ɛma obi ho yɛ no hyew, ne nea ɛkeka ho) na wɔko tia. Sɛ ɔhɔho ntuafo a ɔte saa ba a, saa mast cells yi yi nnuru te sɛ `(Histamine)` fi adi. Saa `(Histamine)` yi na ɛde saa akisikuru ho sɛnkyerɛnne (te sɛ kɔkɔɔ, ɛyaw, ɛho ahonhon) ba. Mpɛn pii no, mast cells gyae saa `(Histamine)` yi gyae bere a wɔayɛ adwuma no. Nanso, wɔ obi a ɔwɔ `(Systemic Mastocytosis)` nipadua mu no, saa mast nkwammoaa a ɛnteɛ yi nyin dodo na ɛma `(Histamine)` kɔ so. Ɛte sɛ nea obi gyaw nsu tap bi wɔ so na afei ne werɛ fii sɛ obedum. Eyi nti na yɛkɔ so nya sɛnkyerɛnne ahorow a ɛte sɛ akisikuru no. Ɛnyɛ yɛn honam ani nko na ebetumi aka, na mmom yɛn mu akwaa te sɛ yɛn mmerɛbo, yɛn akyi berɛmo, ne yɛn dwensɔtwaa nso. Wɔkyerɛ sɛ saa tebea yi a wɔfrɛ no ``Systemic Mastocytosis,'' no ka nnipa 100,000 biara mu 13 pɛ wɔ wiase. Ɛno kyerɛ sɛ ɛntaa nsi koraa. Nanso, nnipa a wɔwɔ saa tebea yi wɔ asiane kɛse mu sɛ wobenya anaphylaxis ( akisikuru a emu yɛ den, mpofirim, a ɛde wɔn nkwa to asiane mu). Ɛntaa mma koraa sɛ ``Systemic Mastocytosis'' nso betumi adan kokoram. Nnuruyɛfo ntumi nsa tebea yi koraa. Nanso, ayaresa ahorow bi wɔ hɔ a ebetumi aboa wo ma woadi wo yare no ho sɛnkyerɛnne so na woabɔ bra sɛnea ɛsɛ.

Dɛn ne `(Systemic Mastocytosis)` ahorow atitiriw?

``Systemic Mastocytosis`` ahorow atitiriw asia na ɛwɔ hɔ. Wɔka yɛn wɔ akwan horow so. Mpɛn pii no, dodow a mast cells a ɛnyɛ ne kwan so wɔ wo nipadua mu no, dodow no ara na ɛda adi sɛ wubenya nsɛnnennen. Momma yɛnhwɛ nea saa ahorow no yɛ: 1. Ɔkwan a ɛnyin brɛoo (``Indolent systemic mastocytosis``):

• Eyi ne ɔkwan a wɔtaa de di dwuma. Te sɛ dua a enyin nkakrankakra no, ebetumi adi mfe pii ansa na yare no ho sɛnkyerɛnne ada adi. Mast cells a ɛnyɛ ne kwan so dodow kɔ soro bere a bere kɔ so no. Eyi betumi de sɛnkyerɛnne ahorow aba honam ani, mmerɛbo , akyi berɛmo , ne yafunu mu nhyehyɛe (i.e., dwensɔtwaa) mu.

2. Ɔkwan a ɛkɔ akyiri kakra (`Systemic smoldering mastocytosis`):

• Wɔ saa su yi mu no, mast cells a ɛnyɛ ne kwan so boaboa ano pii wɔ mmerɛbo ne akyi berɛmo mu. Bere kɔ so no, eyi betumi ama mmerɛbo anaa akyi berɛmo no ahonhon. Susuw ho te sɛ nea ɛba bere a nnipa ahyɛ ofie ketewa bi mu ma dodo no.

3. Ɔkwan a ɛne mogya mu nyarewa afoforo ba (`Systemic mastocytosis with associated hematologic neoplasm`):

• Nnipa a wɔwɔ saa yi betumi anya tebea afoforo a ɛfa mogya ho, te sɛ myeloproliferative neoplasms ne myelodysplastic syndrome . Saa ɔkwan yi ka nnipa bɛyɛ baanum biara mu biako a wɔwɔ Systemic Mastocytosis.

4. Ɔkwan a emu yɛ den (`Aggressive systemic mastocytosis`):

• Eyi yɛ aniberesɛm kakra efisɛ ebetumi aka nnompe mu ntini (baabi a wɔyɛ mogya mu nkwammoaa) ne nnompe. Mast cells a ɛnteɛ no tumi nyin wɔ nnompe no mu, na ɛma ɛyɛ mmerɛw na ɛma ɛyɛ mmerɛw sɛ ebebubu.

5. Ɔkwan a ɛdannan mogya mu kokoram (`Mast cell leukemia`):

• Ɛntaa mma koraa sɛ Systemic Mastocytosis betumi abɛyɛ tebea bi a wɔfrɛ no Mast Cell Leukemia . Eyi yɛ Acute Myeloid Leukemia (AML) bi .

6. Akisikuru no su (`Mast cell sarcoma`): .

• Eyi ne bere a akisikuru (akisikuru) a efi mast nkwammoaa a ɛnteɛ mu ba sɛe ntini ahorow a ɛwɔ nipadua no mu no. Nnipa kakraa bi pɛ a wɔwɔ ``Systemic Mastocytosis`` nya saa tebea yi a wɔfrɛ no ``Mast cell sarcoma``.

Dɛn ne yare yi (Systemic Mastocytosis) ho sɛnkyerɛnne?

Nsɛnkyerɛnne no betumi ayɛ soronko wɔ onipa biara ho. Egyina baabi a mast cells a ɛnteɛ no dɔɔso kɛse wɔ nipadua no mu so. Sɛ nhwɛso no, sɛ wunya mast cells pii wɔ wo yafunu mu a, wubetumi anya akuru ne yafunu mu yaw. Afei nso, sɛ mast cells boaboa ano wɔ wo nnompe mu a, ebetumi aka mogya mu nkwammoaa a wɔyɛ no. Nsɛnkyerɛnne a ɛtaa ba no bi ni:

• Anemia ( mogya a enni nipadua mu ) .
• Nnompe mu yaw
• Mogya a ɛtɔ dodo (sɛ nhwɛso no, mogya pii a efi kuru ketewaa bi mu) .
• Ɔbrɛ a wɔte nka bere nyinaa (` Ɔbrɛ` ) .
• Wora a ayɛ fĩ
• Koma a ɛbɔ denneennen
• Hives (ɛyɛ akisikuru, akisikuru a ɛkɔ soro wɔ honam ani) .
• Wora a ɛyɛ wo yaw
• Adwene mu nsakrae anaa adwenemhawadwenemhaw `) .
• Nsonsonoe a ɛyɛ tumm a ɛyɛ yaw wɔ honam ani (`Urticaria pigmentosa`) .

Ɛtɔ da bi a, nnipa a wɔwɔ Systemic Mastocytosis betumi anya saa sɛnkyerɛnne ahorow yi pii prɛko pɛ. Nnuruyɛfo frɛ eyi "mastocytosis ntua" anaa "flare."

Nea ɛho hia: Ɛnyɛ obiara a ɔwɔ saa sɛnkyerɛnne ahorow yi na ɔwɔ `(Systemic Mastocytosis).` Nanso, sɛ wowɔ saa sɛnkyerɛnne ahorow yi pii prɛko pɛ, na ɛkɔ so a, akyinnye biara nni ho sɛ ɛsɛ sɛ wokɔ oduruyɛfo nkyɛn kɔhwehwɛ afotu.

Dɛn nti na eyi `(Systemic Mastocytosis)` ba?

Nhwehwɛmu ada no adi sɛ nnipa a wɔwɔ `(Systemic Mastocytosis)` no mu bɛyɛ 80% wɔ `KIT gene variant` . Saa `KIT` gene yi boa nkwammoaa ahorow bi a ɛwɔ yɛn nipadua mu, titiriw mogya nkwammoaa ne mast cells, ma wonyin. Saa awosu mu nkwaadɔm a ɛyɛ soronko yi ba bere a wɔanyinsɛn yɛn wɔ awotwaa mu akyi. Eyi kyerɛ sɛ ɛnyɛ agyapade.

Dɛn ne nneɛma a ɛma yare no mu yɛ den anaasɛ ɛkanyan (`Triggers`)?

Nneɛma pii betumi akanyan ``Systemic Mastocytosis.`` ho sɛnkyerɛnne.`` Ɛnyɛ obiara na nneɛma koro na ɛka no. Nanso, nneɛma kakraa bi wɔ hɔ a ɛtaa ba:

• Nsaden
• Nnuan a ɛyɛ nnuhwam
• Nnuru bi: Sɛ nhwɛso no , NSAID ( nnuru a wɔde kum ɛyaw), nnuru a ɛma ntini mu dwo, ne nnuru a wɔde kum nipadua no.
• Apɔw-mu-teɛteɛ ne apɔw-mu-teɛteɛ ( ebinom nkutoo de) .
• Nkoekoemmoa a wɔka wɔn (titiriw nwansena, ntɛtea, ne ntɛtea) .
• Honam fam anaa nkate fam nhyɛso
• Wo honam ani a wobɛpetepete anaasɛ wobɛbɔ wo honam ani
• Nsakrae a ɛba ɔhyew mu mpofirim (sɛ nhwɛso no, mpofirim a wohuruw kɔ nsuonwini mu) .

Sɛ wowɔ saa yare yi a, ɛho hia paa sɛ wubisa wo duruyɛfo no nea ɛyɛ wo mmerɛwyɛ ne nea ɛsɛ sɛ wokwati.

Wobɛyɛ dɛn ahu yare yi pɛpɛɛpɛ? (Nyare a Wɔahu) .

Sɛ wokɔ oduruyɛfo nkyɛn a, ade a edi kan a wɔbɛyɛ ne sɛ wɔbɛyɛ wo nipadua mu nhwehwɛmu. Wobebisa wo yare no ho sɛnkyerɛnne ne nyarewa biara a woadi kan anya ho asɛm. Afei, ebia wɔbɛyɛ nhwehwɛmu a edidi so yi mu biako anaa nea ɛboro saa de ahu sɛ wowɔ Systemic Mastocytosis anaa:

• Mogya mu nhwehwɛmu: Eyi hwɛ tryptase dodow a ɛwɔ wo mogya mu. Tryptase yɛ enzyme titiriw bi a mast cells gyae bere a wɔyɛ wɔn ade wɔ nneɛma a ɛyɛ ananafo ho no.
• Nnompe mu nhwehwɛmu: Hwɛ sɛ nnompe asɛe anaa.
• Bone marrow biopsy: Eyi hwehwɛ sɛ wobɛfa wo nnompe mu ntini ketewaa bi na woasɔ ahwɛ sɛ ebia mast cells bi a ɛnyɛ ne kwan so wɔ mu, ne dodow a ɛwɔ mu.
• Awosu mu nhwehwɛmu: Hwɛ sɛ nkwaadɔm mu nsakrae bi wɔ `KIT` awosu mu abɔde a yɛadi kan aka ho asɛm no mu anaa.

Ɔkwan bɛn so na wɔsa?

Nnuruyɛfo sa Systemic Mastocytosis denam sɛnkyerɛnne ne nsɛnnennen a wodi so no so. Sɛ nhwɛso no, sɛ wowɔ yafunu mu acid pii a, ebia wɔbɛkyerɛw nnuru a esiw H2 ano (aduru bi a ekum asikre). Sɛ mogya a ɛtɔ kɔ nipadua mu no fi yare no a, wɔbɛsa. Ayaresa atitiriw no bi ni:

• Nnuru a ekum histamine: Ɛtew honam ani yare te sɛ honam ani yare ne kɔkɔɔ so.
• Corticosteroids: Ɛtew ɔfe ne ɔfe a ɛwɔ nipadua no mu so.
• Bisphosphonates: Sɛ nnompe yɛ mmerɛw a, ɛboa ma ɛyɛ den.
• Ayaresa a wɔde wɔn ani asi so: Eyi hwehwɛ sɛ wɔde wɔn ani si protein pɔtee bi a wohu wɔ mast cells a ɛnyɛ ne kwan so no so.
• Nnuru a wɔde sa yare: Sɛ Systemic Mastocytosis adan kokoram nkutoo a, na wɔde eyi di dwuma.
• Splenectomy: Sɛ ahurututu no ahonhon kɛse a, wobetumi ayɛ oprehyɛn ayi afi hɔ.
• Nnompe mu ntini a wɔde si ananmu: Wɔyɛ eyi ma nnipa a wɔwɔ yare a emu yɛ den yiye, anaa wɔn a wɔwɔ yare a etwa to no.

Kae: Sɛ wowɔ Systemic Mastocytosis a, ɛsɛ sɛ wokura EpiPen bere nyinaa. EpiPen yɛ aduru a wobetumi de adi dwuma ntɛm ara sɛ obi ho yɛ no hyew kɛse te sɛ Anaphylaxis a yɛadi kan aka ho asɛm no.

So wontumi nsiw saa ``Systemic Mastocytosis'' yi ano?

Sɛnea yɛadi kan aka ho asɛm no, ``Systemic Mastocytosis`` fi awosu mu nsakrae. Enti, ɛrentumi nyɛ yiye sɛ wobesiw ano. Nanso, wubetumi adi yare no so denam ``nneɛma a ɛkanyan`` a ɛma sɛnkyerɛnne ahorow no yɛ kɛse a wobɛkwati so.

Daakye bɛn na obi a ɔwɔ `(Systemic Mastocytosis)` wɔ?

Eyi gyina `(Systemic Mastocytosis)` ko a wowɔ so ankasa. Nnipa a wɔwɔ ɔkwan a ɛyɛ brɛoo (`Indolent systemic mastocytosis`) no taa tumi di yare no so yiye denam ayaresa so. Wobetumi atra ase wɔ ɔkwan a ɛfata so. Nanso, ebia nnipa a wɔwɔ yare afoforo a emu yɛ den no nkwa nna yɛ tiaa kakra. Nanso, wobetumi de aduru bi a wɔfrɛ no ``Bone marrow transplant`` asa `(Systemic Mastocytosis)` yare koraa. Nanso, nnipa a yare no mu yɛ den paa nkutoo na nnuruyɛfo de saa ayaresa no ma.

Mɛyɛ dɛn ahwɛ me ho bere a me ne `(Systemic Mastocytosis)` te?

Nim a wubehu nea ɛkanyan wo sɛnkyerɛnne ahorow no na woakwati no ne ade a ɛho hia sen biara a wubetumi ayɛ bere a wo ne Systemic Mastocytosis te no. Sɛ wowɔ saa tebea yi a, hwɛ nneɛma yi so yiye:

• Fa EpiPen ka wo ho bere nyinaa. Sa akisikuru a emu yɛ den ntɛm.
• Hu nea ɛkanyan wo ankasa na kwati sɛnea wubetumi biara.
• Di adwennwen ho dwuma. Fa adwendwene anaa akwan foforo a wɔfa so de adwene si nneɛma so di dwuma.
• Hyɛ aduruyɛ ho kɔkɔbɔ abɔso a wɔakyerɛw nnuru a wuntumi nnom. Eyi ho hia yiye efisɛ ɛboa nnuruyɛfo ma wohu wo tebea no sɛ wuntumi nkasa wɔ tebea a egye ntɛmpɛ mu a.

Bere bɛn na ɛsɛ sɛ mekɔ oduruyɛfo nkyɛn?

Sɛ wowɔ Systemic Mastocytosis a, ɛho behia sɛ wokɔ wo duruyɛfo nkyɛn kɔhwɛ wo daa. Afei nso, hwɛ hu sɛ wobɛka akyerɛ wo duruyɛfo sɛ wunya yare no ho sɛnkyerɛnne foforo bi, anaasɛ ɛte sɛ nea wo sɛnkyerɛnne no bi reyɛ kɛse a.

Nsɛm bɛn na ɛsɛ sɛ mibisa oduruyɛfo no?

Esiane sɛ Systemic Mastocytosis yɛ yare a ɛntaa nsi nti, ebia wubenya nsɛmmisa pii. Nsɛmmisa bi a ɛsɛ sɛ wubisa wo duruyɛfo ni:

• ``Systemic Mastocytosis'' bɛn na mewɔ?
• Nsɛnkyerɛnne afoforo bɛn na ebetumi akyerɛ sɛ me tebea no reyɛ kɛse?
• Ayaresa bɛn na ɛwɔ hɔ ma me su no?
• So ayaresa no beyi me yare no ho sɛnkyerɛnne no afi hɔ koraa?
• So nnuruyɛ mu sɔhwɛ ahorow bi wɔ hɔ a metumi de me ho ahyɛ mu?

Sɛ wohu sɛ wowɔ Systemic Mastocytosis a, ɛyɛ osuahu a ɛsakra asetra. Ɛsɛ sɛ wokɔ wo duruyɛfo nkyɛn daa, woyɛ wo mogya mu nhwehwɛmu, na wusua sɛnea wubehu nneɛma a ɛkanyan wo na woakwati. Tebea yi a wobɛtra ase no yɛ nsakrae kɛse. Enti, kɔ so ne wo duruyɛfo no nnya abusuabɔ pa. Wɔwɔ hɔ sɛ wɔbɛboa wo. Wɔbɛma wo nsɛm ne akuw a wɔboa wo a wobetumi aboa wo. Eyinom bɛma woatumi asi w’akwahosan ho gyinae.

Nea etwa to no, nneɛma a ɛsɛ sɛ wokae (Take-Home Message) .

Okay, enti yɛaka `(Systemic Mastocytosis)` ho asɛm pii, ɛnte saa? Kae sɛ, ɛwom sɛ eyi yɛ yare a ɛntaa nsi de, nanso ɛho hia yiye sɛ wohwɛ so yiye.

Nea ɛho hia sen biara ne sɛ wobɛkɔ oduruyɛfo nkyɛn sɛ wowɔ saa sɛnkyerɛnne ahorow yi a. Sɛ wohu yare no ntɛm a, wobetumi afi ase sa yare na wɔatumi asiw yare no ho sɛnkyerɛnne so.

Ɛnyɛ wo nko ara. Wo nnuruyɛfo, w’abusuafo, ne wo nnamfo wɔ hɔ sɛ wɔbɛboa wo. Sɛ wuhu nsɛm a ɛfata na wudi wo duruyɛfo afotu akyi a, wubetumi ne tebea yi atra ase yiye. Yɛ komm na susuw nneɛma pa ho!