So wo ho yɛ wo yaw bere nyinaa? So ɛdan kɔkɔɔ? Momma yensua yare a wɔntaa nhu (Systemic Mastocytosis) yi ho ade .

So wo nipadua nyinaa nso yɛ wo yaw bere ne bere mu? So w’anim ne wo kɔn yɛ kɔkɔɔ mpofirim na ɛte sɛ nea woreyɛ hyew? So wo yafunu yɛ wo yaw, wo ti so yɛ wo yaw, na wo koma tu mmirika? Sɛ mpo wususuw sɛ saa nneɛma yi yɛ akisikuru ara kwa a, ɛtɔ mmere bi a ebia tebea foforo bi wɔ eyi akyi, nea yɛntee ho asɛm pii. Ɛnnɛ yɛreka yareɛ baako a ɛntaa nsi a ɛte saa, Systemic Mastocytosis ho asɛm. Mma ɛnhaw wo, momma yɛnte eyi ase kɛkɛ.

Dɛn ne nhyehyɛe mu mastocytosis?

Sɛ yɛbɛka no tiawa a, eyi yɛ yare a efi nkwammoaa bi a wɔfrɛ no ‘mast cells’ a ɛwɔ yɛn nipadua mu a ɛkɔ soro wɔ ɔkwan a ɛnteɛ so na ɛba. Mast cells yɛ mogya mu nkwammoaa fitaa bi. Susuw saa mast cells yi ho sɛ asraafo wɔ yɛn nipadua mu ahoɔden a ɛko tia nyarewa no mu. Wɔn adwuma titiriw ne sɛ wɔbɛbɔ yɛn ho ban afi nyarewa ho na wɔaboa yɛn ma yɛanya ayaresa bere a yɛapira no . Nanso wɔ saa yare yi mu no, saa mast cells yi boaboa ano boro so na ɛnyɛ ne kwan so wɔ akwaa ahorow te sɛ yɛn honam ani, nnompe mu ntini , nnompe, ne dwensɔtwaa mu. Dɛn na ɛba saa bere no? Esiane biribi a ɛkanyan no nti, mast cells a ɛboro so yi yɛ adwuma mpofirim na ɛma nnuru pii te sɛ ‘histamine’ kɔ nipadua no mu. Ɛno ne bere a sɛnkyerɛnne ahorow a madi kan aka ho asɛm, te sɛ nsu a ɛyɛ kurukuruwa, ɛyaw, ne eczema , da adi.

Dɛn na ɛde yare yi ba?

Nnipa pii susuw sɛ ebia eyi yɛ awosu mu yare anaa. Nanso eyi ntaa nyɛ yare a wonya fi awo mu. Mpɛn pii no, ɛba wɔ awosu mu abɔde bi a wɔfrɛ no KIT a ɛwɔ yɛn mast cells mu no mu.Nsakrae a ɛba kwa (mutation) na ɛde ba. Saa awosu mu abɔde yi boa ma wɔyɛ protein a ɛhwɛ nkwammoaa nyin so. Enti sɛ eyi sesa a, mast cells fi ase nyin a wontumi nni so. Ɛwom sɛ saa sɛnkyerɛnne ahorow yi betumi ada adi wɔ mfe biara mu de, nanso ɛtaa ba nnipa a wɔn mfe akɔ anim mu, titiriw bere a wɔadi fi mfe 40 kosi 50 .

Nneɛma a ɛkanyan a ɛma sɛnkyerɛnne ahorow no mu yɛ den

Ɛnyɛ obiara a ɔwɔ saa yare yi na onya yare no ho sɛnkyerɛnne koro. Afei nso, nneɛma a ɛkanyan yare no ho sɛnkyerɛnne no betumi ayɛ soronko wɔ onipa biara ho. Nanso, wɔakyerɛw nneɛma a ɛtaa kanyan obi no bi wɔ pon a ɛwɔ ase ha no mu. Ɛho hia sɛ wohu eyinom. Ɛho hia nso sɛ yɛkae sɛ ɛtɔ mmere bi a, yare no ho sɛnkyerɛnne betumi ada adi a ntease pɔtee biara nni mu.

Dɛn ne yare yi ahorow ne ne sɛnkyerɛnne atitiriw?

Systemic Mastocytosis ahorow atitiriw anum na ɛwɔ hɔ. Wo duruyɛfo bɛsɔ wo ahwɛ na wakyerɛ wo type a wowɔ.

• Indolent Systemic Mastocytosis (ISM): Eyi ne nea abu so sen biara na ɛnyɛ den koraa. Yare no kɔ so nkakrankakra na ɛnsɛe akwaa ahorow kɛse.
• Smoldering Systemic Mastocytosis (SSM): Ɛyɛ den kakra sen ISM. Nsɛnkyerɛnne no mu yɛ den kɛse na bere kɔ so no, ebetumi aka akwaa ahorow.
• Systemic Mastocytosis with an Associated Hematological Neoplasm (SM-AHN): Wɔ eyi mu no, yare foforo a efi mogya mu (e.g. leukemia ) ka mastocytosis ho.
• Aggressive Systemic Mastocytosis (ASM): Saa ɔkwan yi kɔ so ntɛmntɛm na ebetumi asɛe akwaa te sɛ mmerɛbo, akyi berɛmo, ne nnompe.
• Mast Cell Leukemia (MCL): Eyi ne nea ɛntaa nsi na emu yɛ den sen biara. Mast cells pii wɔ nnompe mu ntini ne mogya mu.

Nsɛnkyerɛnne a wubehu no gyina yare ko a wowɔ ne baabi a mast cells a ɛnyɛ ne kwan so no dɔɔso kɛse wɔ wo nipadua mu no so. Nsɛnkyerɛnne a ɛtaa ba no bi ni:

• Itching, rash , nsuo a ɛyɛ fĩ
• Yafunu mu yaw, akisikuru, akisikuru
• Akisikuru anaa ɔfe
• Tiyɛ ne ɔbrɛ
• Koma a ɛbɔ ntɛmntɛm
• Mogya mmoroso a ɛba fam, adwenemhaw, adwene a ɛyera
• Mmɛn, akyi berɛmo, ne ntini a ɛma mogya tu no ho ahonhon
• Mogya a ɛtɔ kɔ nipadua mu ne mogya a ɛtɔ
• Nnompe a ɛyɛ mmerɛw (Osteoporosis) .
• Adwene mu no, tebea horow te sɛ ɛyɛ den sɛ obi de n’adwene besi biribi so, dadwen a ɔtaa ba, anaa adwenemhaw.

Ɔkwan bɛn so na wobetumi ahu yare no? (Nyare a Wɔahu) .

Wo duruyɛfo bedi kan abisa wo wo yare no ho sɛnkyerɛnne na wayɛ wo nipadua mu nhwehwɛmu. Afei, ebia wɔbɛkamfo nhwehwɛmu pii akyerɛ de akyerɛ sɛ wɔahu yare no:

• Skin biopsy: Woyi honam ani fã ketewaa bi fi baabi a ayɛ no na wɔde microscope hwehwɛ mu hwɛ sɛ mast cells pii wɔ hɔ anaa.
• Bone Marrow Biopsy: Mogya ho ɔyaresafo de ade a ɛyɛ tratraa di dwuma de fa wo nnompe mu ntini ketewaa bi na ɔhwehwɛ mu.
• Mogya ne nsu mu nhwehwɛmu:Wobetumi de mogya dodow a edi mũ (CBC) nso ahwɛ sɛ ebia tebea afoforo te sɛ mogya a ɛtɔ kɔ nipadua mu no wɔ hɔ anaa.
• Awosu mu Nhwehwɛmu: Wɔsɔ mogya anaa ntini mu nhwehwɛmu hwɛ sɛ nsakrae bi aba KIT awosu mu nkwaadɔm a yɛadi kan aka ho asɛm no mu anaa.
• Mfoniniyɛ mu nhwehwɛmu: X-ray, ultrasound, ne CT scan betumi aboa ma wɔahu nnompe mu mmerɛwyɛ ne akwaa te sɛ mmerɛbo ne akyi berɛmo a ɛho ahonhon.
• Endoscopy anaa Colonoscopy: Wɔde afiri a ɛyɛ tratraa a mfoninitwa afiri abata ho di dwuma de hwehwɛ yafunu no mu anaa dwensɔtwaa mu hwɛ sɛ ebia biribi a ɛnteɛ te sɛ akuru wɔ hɔ anaa.

Dɛn ne ayaresa ahorow no?

Oduruyɛfo no begyina wo yare no ho sɛnkyerɛnne, akwaa a ɛka wo, ne yare ko a wowɔ so akyerɛ ayaresa nhyehyɛe no.

• Nnuru a ɛko tia akisikuru: Nnuru yi boa ma wodi nneɛma te sɛ honam ani yaw, kɔkɔɔ, ne yafunu a ɛyɛ yaw so.
• Epinephrine: Hormone a wɔde di dwuma bere a obi ho yɛ no yaw kɛse (anaphylaxis).
• Proton Pump Inhibitors: Di yafunu mu acid so na ɛtew nneɛma te sɛ yafunu mu ɔfe so.
• Mast Cell Stabilizers: Nnuru a ɛma histamine a efi mast cells mu no gyae.
• Steroids: Ebetumi aboa wɔ honam ani ne ɔhome mu sɛnkyerɛnne ahorow ho.
• Targeted Therapy: Nnuru a wɔde si awosu mu abɔde anaa protein pɔtee bi a ɛka yare no ho. Ebia eyinom ho behia ama nnipa a wɔwɔ yare no ho sɛnkyerɛnne a emu yɛ den.
• Nnuru a wɔde sa yare: Ebia ayaresa yi ho behia wɔ nsɛm a yare no retrɛw ntɛmntɛm na ɛresɛe akwaa ahorow kɛse mu.
• Stem Cell Transplant: Ayaresa a wosusuw ho wɔ nsɛm a ɛntaa nsi koraa mu.

Systemic Mastocytosis, mast cells, honam ani akisikuru, anaphylaxis, anaphylaxis, KIT awosu mu abɔde, histamine