So wo nnompe mu ntini nso ma ntini a ɛyɛ nhama? Momma yensua myelofibrosis ho ade!

So woate yare bi a wɔfrɛ no myelofibrosis ho asɛm pɛn? Nokwarem no, ɛyɛ mogya mu kokoram a ɛntaa nsi koraa. Sɛ yɛbɛka no tiawa a, ɛyɛ bere a wɔde nhama a ɛyɛ akuru si nnompe mu ntini , a ɛyɛ wo nnompe no fã a ɛyɛ mmerɛw na ɛte sɛ sponge no ananmu no . Ɛsan nso yɛ mogya mu kokoram a ɛtra hɔ kyɛ, na ɛka kuw bi a wɔfrɛ no myeloproliferative disorders ho. Myeloproliferative disorders yɛ bere a wɔyɛ mogya mu nkwammoaa pii dodo wɔ nnompe mu ntini mu, baabi a wɔyɛ mogya mu nkwammoaa no.

Dɛn ankasa ne myelofibrosis?

Momma yɛnhwɛ eyi mu nkɔ akyiri. Nkwammoaa a ɛma mogya yɛ wo nnompe mu . Akyiri yi saa ntini mu nkwammoaa yi nyin bɛyɛ mogya mu nkwammoaa kɔkɔɔ, mogya mu nkwammoaa fitaa, ne mogya mu nkwammoaa kɔkɔɔ. Nanso wɔ obi a ɔwɔ myelofibrosis mu no, nsakrae bi ba awosu mu nneɛma anaa DNA a ɛwɔ saa ntini mu nkwammoaa yi biako mu no mu. Eyi ma nkwammoaa no dan kokoram nkwammoaa a asɛe. Afei saa nkwammoaa a asɛe yi mu paapae na ɛde nkwaadɔm mu nsakrae no kɔ nkwammoaa foforo a ɛyɛ no nyinaa mu.

Bere kɔ so no, kokoram nkwammoaa a ɛnteɛ yi dɔɔso pii. Nkwammoaa yi bi ma nnompe mu ntini mu hyehye. Saa ɔfe yi na ɛma akuru ntini a madi kan aka ho asɛm no yɛ. Enti, esiane saa akuru yi ne kokoram nkwammoaa a ɛboro so nti, nnompe mu ntini no ntumi nyɛ mogya mu nkwammoaa a ɛte apɔw.

Dɛn ne myelofibrosis ahorow atitiriw?

Myelofibrosis ahorow atitiriw abien na ɛwɔ hɔ.

• Primary myelofibrosis: Eyi ne nea ɛba ara kwa, a ɛne yare foforo biara nni abusuabɔ biara.
• Myelofibrosis a ɛto so abien: Mogya mu yare foforo na ɛde eyi ba. Sɛ nhwɛso no, ebetumi afi nyarewa te sɛ primary thrombocytosis anaa polycythemia vera mu aba. Saa ɔkwan a ɛto so abien yi yɛ ayarefo a wɔahu wɔn no mu 10% kosi 20%.

Yare yi a wɔfrɛ no Myelofibrosis abu so dɛn?

Myelofibrosis yɛ tebea a ɛntaa nsi koraa ankasa. Sɛ nhwɛso no, wɔ United States no, wɔbɔ amanneɛ sɛ yare yi ba nnipa 100,000 biara mu bɛyɛ 1.5 afe biara. Ebetumi aka obiara, ɛmfa ho mfe a wadi. Nsonsonoe biara nni mfe a obi adi, nanso ɛda adi sɛ wobehu wɔ nnipa a wɔadi boro mfe 50. Sɛ myelofibrosis ba mmofra nkumaa mu a, wɔtaa hu ansa na wɔadi mfe 3.

Nkɛntɛnso bɛn na myelofibrosis betumi anya wɔ wo nipadua so?

Sɛ wɔyɛ mogya mu nkwammoaa wɔ ɔkwan a ɛnteɛ so wɔ saa kwan yi so a, nsɛnnennen ahorow betumi aba. Momma yɛnhwɛ nea wɔyɛ:

• Mogya a ɛtɔ kɔ nipadua mu: Eyi yɛ tebea a mogya mu nkwammoaa kɔkɔɔ nnɔɔso. Sɛnea wunim no, mogya mu nkwammoaa kɔkɔɔ na ɛde mframa pa kɔ nipadua no mu nyinaa. Enti sɛ nkwammoaa kɔkɔɔ nnɔɔso a, nipadua no mu ntini nnya mframa pa a ɛdɔɔso. Eyi betumi de sɛnkyerɛnne ahorow te sɛ ɔbrɛ, mmerɛwyɛ, ne ahome a ɛyɛ tiaa aba .

• Thrombocytopenia: Eyi yɛ tebea a mogya nkwammoaa kakraa bi na ɛwɔ mu. Ntini mu nkwammoaa a ɛma mogya tu ne nea ɛboa ma mogya tu bere a obi apira no. Enti sɛ mogya nkwammoaa a ɛwɔ wo mogya mu no sua a, mogya betumi atu wo nipadua na ayɛ mmerɛw kɛse .

• Splenomegaly: W’ahurututu no hwɛ mogya mu nkwammoaa dodow so na eyi mogya mu nkwammoaa kɔkɔɔ a asɛe fi wo nipadua mu. Enti sɛ woyɛ mogya mu nkwammoaa a ɛnteɛ pii dodo a, ɛsɛ sɛ wo dwonku yɛ adwumaden. Ebetumi ayɛ kɛse. Sɛ wo dwonku sõ a, ebia wobɛte nka sɛ wo yafunu no benkum so atifi ayɛ ma anaasɛ wo ho nyɛ wo dɛ .

• Extramedullary hematopoiesis: Eyi ne nkwammoaa a ɛyɛ mogya a ɛba wɔ nnompe mu ntini no akyi , wɔ nipadua no afã afoforo. Saa nkwammoaa yi tumi hyehyɛ mmeae te sɛ ahurututu, yafunu mu ntini, akyi berɛmo, amemene, anaa ntini a ɛma mogya tu. Saa nkwammoaa yi tumi bom yɛ akisikuru , a ebetumi akɔhyɛn akwaa afoforo so anaasɛ asiw wɔn dwumadi kwan.

• Portal hypertension: Eyi yɛ mogya mmoroso a ɛkɔ soro wɔ ntini a ɛde mogya fi wo dwonku mu kɔ wo mmerɛbo mu no mu. Ɛda adi kɛse sɛ eyi fi ntini no a ɛsɛe esiane mogya a ɛba nipadua no akyi a yɛadi kan aka ho asɛm no nti.

Ade foforo ne sɛ wɔ nnipa a wɔwɔ myelofibrosis titiriw no mu bɛyɛ 12% fam no, tebea no betumi abɛyɛ mogya mu kokoram a emu yɛ den yiye a wɔfrɛ no acute myeloid leukemia .

Dɛn na ɛde myelofibrosis ba?

Nyansahufo da so ara nnim nea ɛde myelofibrosis ba ankasa. Nanso wɔahu sɛ ɛne nsakrae a ɛba awosu mu nkwaadɔm bi DNA mu wɔ abusuabɔ . Protein bi a wɔfrɛ no Janus-associated kinases (JAKs) na ɛka ho. Saa JAKs protein ahorow yi hwɛ mogya mu nkwammoaa a wɔyɛ wɔ nnompe mu ntini mu no so, na ɛde nsɛnkyerɛnne kɔma nkwammoaa no ma wɔkyekyɛ na wonyin. Sɛ saa JAKs protein ahorow yi yɛ adwuma boro so a, wobetumi ayɛ mogya mu nkwammoaa pii dodo anaasɛ kakraa bi.

Nnipa a wɔwɔ myelofibrosis no mu 60% kosi 65% wɔ nkwaadɔm mu nsakrae wɔ JAK2 awosu mu abɔde mu . 5% kosi 10% foforo .Nsakrae bi wɔ myeloproliferative leukemia (MPL) awosu mu abɔde mu nkwaadɔm mu. Afei nso, wohu nkwaadɔm mu nsakrae bi a wɔfrɛ no Calreticulin (CALR) wɔ ayarefo a wɔwɔ myelofibrosis no mu 20% kosi 25% mu.

Hena na ɔwɔ asiane kɛse ma eyi?

Ebia wowɔ asiane kɛse mu sɛ wubenya myelofibrosis esiane nneɛma a edidi so yi nti:

• Sɛ woadi boro mfe 50 a .
• Sɛ wowɔ mogya mu yare bi a wɔfrɛ no primary thrombocytosis anaa polycythemia vera a.
• Sɛ woanya mframa a ɛma ionizing anaa petrochemicals te sɛ benzene ne toluene a.

Dɛn ne myelofibrosis ho sɛnkyerɛnne ahorow?

Myelofibrosis yɛ yare a ɛkɔ so nkakrankakra , enti ebia worennya sɛnkyerɛnne biara mfe pii. Ayarefo bɛyɛ nkyem abiɛsa mu biako nnya yare no ho sɛnkyerɛnne biara wɔ mfiase.

Sɛ sɛnkyerɛnne ahorow no ba ampa a, nea ɛtaa ba ne ɔbrɛ a emu yɛ den (esiane mogya a ɛtɔ kɔ nipadua mu nti) ne akyi berɛmo a ɛyɛ kɛse . Nsɛnkyerɛnne no nso betumi ayɛ:

• Adwumaden
• Huraeɛ
• Ɛyɛ akisikuru
• Wora a ɛyɛ mmerɛw
• Mu duru a ɛso tew
• Anadwo fifiri a ɛba
• Nnompe anaa nkwaa mu yaw
• Nyarewa a wɔtaa nya
• Akisikuru anaa mmerɛbo a ayɛ kɛse
• Mogya a ɛtwetwe a wontumi nkyerɛkyerɛ mu
• Mogya a ɛtɔ anaasɛ akuru a ɛyɛ soronko
• Ntini a ɛwɔ yafunu ne aduan mu a ɛtrɛw (ntini yi betumi apae na mogya atu).

Ɔkwan bɛn so na wobetumi ahu yare yi?

Oduruyɛfo, titiriw oncologist, bɛhwehwɛ wo nipadua mu, abisa w’ayaresa ho abakɔsɛm, na wabisa wo mprempren sɛnkyerɛnne ahorow ho asɛm. Wɔbɛhwɛ nso sɛ akyi berɛmo no ayɛ kɛse na mogya a ɛtɔ kɔ nipadua no mu ho sɛnkyerɛnne bi wɔ hɔ anaa.

Afei, wɔyɛ nhwehwɛmu ahorow de yi tebea afoforo fi mu na ɛkyerɛ sɛ ebia wowɔ myelofibrosis anaa.

Mogya mu nhwehwɛmu

• Mogya dodow a edi mũ (CBC): Eyi kyerɛ nkwammoaa ahorow dodow a ɛwɔ wo mogya mu. Sɛ wo mogya mu nkwammoaa kɔkɔɔ no sua sen sɛnea ɛte daa, anaasɛ wo mogya mu nkwammoaa fitaa ne mogya mu nkwammoaa kɔkɔɔ no nyɛ nea ɛfata a, ebetumi ayɛ myelofibrosis ho sɛnkyerɛnne.
• Peripheral blood smear (PBS): Eyi hwɛ sɛnea wo mogya mu nkwammoaa no kɛse, ne nsɛso, ne ne su afoforo hwɛ sɛ ebia ɛnyɛ ne kwan so anaa. Sɛ nkwammoaa a ɛte sɛ nea ɛnteɛ na mogya mu nkwammoaa a ennyinii pii wɔ hɔ a, ebetumi nso ayɛ myelofibrosis ho sɛnkyerɛnne.
• Mogya mu nnuruyɛ mu nhwehwɛmu:Saa nhwehwɛmu ahorow yi susuw nneɛma ahorow a efi w’akwaa mu kɔ mogya no mu no dodow. Eyi betumi ama woahu sɛnea akwaa bi reyɛ adwuma yiye. Nneɛma te sɛ uric acid, bilirubin, ne lactic dehydrogenase a ɛdɔɔso wɔ mogya no mu nso betumi ayɛ myelofibrosis ho sɛnkyerɛnne.

Nnompe mu ntini mu nhwehwɛmu

• Nnompe mu ntini mu nhwehwɛmu: Nea ɛka eyi ho ne sɛ wobɛfa wo nnompe mu ntini ketewaa bi na wode afiri a wɔde hwɛ nneɛma nketenkete ahwɛ mu. Wɔhwehwɛ nkwammoaa no mu hwɛ sɛ wowɔ myelofibrosis anaa.
• Nnompe mu ntini a wɔtwetwe: Eyi hwehwɛ sɛ woyi nsu a ɛwɔ nnompe mu ntini no mu na wɔsɔ hwɛ sɛ ebia myelofibrosis ho sɛnkyerɛnne bi wɔ hɔ anaa.

Ebia ɛho behia sɛ wɔyɛ sɔhwɛ foforo

Ebia ɛho behia sɛ wɔyɛ nhwehwɛmu foforo de si so dua sɛ woahu yare no:

• Awosu mu nkwaadɔm mu nsakrae nhwehwɛmu: Wo duruyɛfo bɛsɔ wo mogya ne nnompe mu nkwammoaa ahwɛ sɛ ebia awosu mu nkwaadɔm mu nsakrae a ɛbata myelofibrosis ho te sɛ JAK2, CALR, ne MPL anaa. Ayaresa ahorow bi de wɔn ani si kokoram nkwammoaa a wɔwɔ JAK2 mutation no so.
• Mfoniniyɛ ho nhyehyɛe: Ebia wo duruyɛfo bɛyɛ ultrasound de ahwɛ sɛ akyi berɛmo no ayɛ kɛse anaa. Ebia wɔbɛyɛ MRI nso de ahwɛ sɛ akuru wɔ nnompe mu ntini no mu anaa. Saa akuru yi betumi ayɛ myelofibrosis ho sɛnkyerɛnne.

Ɔkwan bɛn so na wɔsa myelofibrosis?

Sɛ woankyerɛ yare no ho sɛnkyerɛnne a, ebia worenhia ayaresa. Nanso, sɛ wunhia ayaresa ntɛm mpo a, wo duruyɛfo no bɛkɔ so ahwɛ wo tebea no so.

Jakafi® (ruxolitinib), Inrebic® (fedratinib), ne Vonjo® (pacritinib) yɛ nnuru a U.S. Aduan ne Nnuru Dwumadibea (FDA) apene so . Wɔde sa myelofibrosis a ɛyɛ mmerɛw kosi nea asiane wom kɛse. Nnuru abiɛsa yi nyinaa yɛ adwuma sɛ nnuru a esiw JAK ano . Ɛne sɛ, wɔtew Janus-associated kinase (JAK) sɛnkyerɛnne a ɛyɛ adwuma boro so no so. Ne saa nti, nnuru yi boa ma sɛnkyerɛnne ahorow a ɛbata myelofibrosis ho, te sɛ akyi berɛmo a ɛyɛ kɛse, fifiri a ɛba anadwo, ɛyaw, mu duru a ɛso tew, ne atiridiinini no fi hɔ.

Wɔ nnipa pii fam no, ayaresa botae titiriw ne sɛ wobedi tebea horow a ɛbata myelofibrosis ho, te sɛ mogya a ɛtɔ kɔ nipadua mu ne akyi berɛmo a ɛyɛ kɛse no ho dwuma.

Ayaresa a Wɔde Ma Wɔ Mogya a Wɔyare Mu

Ayaresa ahorow bi wɔ hɔ a wɔde sa mogya a ɛtɔ kɔ nipadua mu te sɛ:

• Androgens: Sɛ obi nom androgens te sɛ danazol a, ebetumi ama mogya mu nkwammoaa kɔkɔɔ a wɔyɛ no ayɛ kɛse.
• Nnuru a ɛma nipadua no tumi ko tia nyarewa: Saa nnuru yi ma wo nipadua mu tumi a ɛko tia nyarewa no tumi ko tia kokoram nkwammoaa, na ɛnam so ma yare no ho sɛnkyerɛnne so tew. Nnuru te sɛ interferon, thalidomide (Thalomid®), ne lenalidomide (Revlimid®) ka saa kuw yi ho. Wobetumi nso de aka glucocorticoids te sɛ prednisone ho.
• Nnuru a wɔde sa yare: Nnuru bi a wɔde sa yare no betumi atew sɛnkyerɛnne ahorow a mogya mu nkwammoaa dodow a ɛkɔ soro ne akyi berɛmo a ɛyɛ kɛse de ba no so. Hydroxyurea ne cladribine yɛ nnuru a ɛte saa.
• Mogya a wɔde ma: Sɛ mogya a ɛtɔ wo mu yɛ den a, mogya a wode bɛma wo daa no betumi ama wo mogya mu nkwammoaa kɔkɔɔ dodow akɔ soro.

Splenomegaly Ayaresa a Wɔde Ma

Wɔde nnuru a esiw JAK ano, nnuru a ɛma nipadua no tumi ko tia nyarewa, ne nnuru a wɔde sa yare di dwuma de di akyi berɛmo a ayɛ kɛse ho dwuma. Wɔ tebea horow a emu yɛ den mu no, ebia ɛho behia sɛ wɔyɛ wo oprehyɛn yi w’akyi berɛmo (splenectomy) anaasɛ wɔyɛ radiation therapy wɔ akyi berɛmo no mu.

Ebia ɛho behia nso sɛ wɔde mframa a wɔde sa yare na wɔasa tebea bi a wɔfrɛ no extramedullary hematopoiesis, a mogya mu nkwammoaa nyin wɔ nnompe mu ntini no akyi no.

So wobetumi asa myelofibrosis koraa?

Ebia allogeneic hematopoietic cell transplantation (HCT) nkutoo ne ɔkwan a wɔbɛfa so asa yare yi. Nanso, eyi yɛ adeyɛ a asiane wom na ɛnyɛ nea ɛfata obiara.

Wɔ saa adeyɛ yi mu no, wɔde nkwammoaa a efi obi a ɔwɔ apɔwmuden a ɔde ma no mu si wo mogya nkwammoaa a ɛnyɛ ne kwan so no ananmu. Ansa na wɔde wo nipadua foforo bɛma wo no, wɔbɛma wo nnuru anaa mframa a wɔde sa wo yare de akunkum wo nkwammoaa a yare no. Afei, nkwammoaa foforo a efi nea ɔde ma no hɔ no betumi agye wo nipadua no dwumadi ahorow.

HCT ayaresa no de asiane kɛse ba sɛ obenya nsɛnnennen . Enti, ɛfata nnipa kuw bi a wɔapaw wɔn nkutoo. Asiane a ɛwɔ hɔ sɛ nsɛnnennen benya no yɛ kɛse mpo ma nnipa a wɔwɔ ayaresa mu tebea afoforo. Sɛ́ ebia wofata ma eyi no gyina nneɛma pii so, te sɛ mfe a woadi, sɛnea wo sɛnkyerɛnne ahorow no mu yɛ den, ne sɛnea ɛbɛyɛ yiye sɛ ayaresa no bedi nkonim.

Sɛ obi ne yare yi te ase a, nkwa nna te dɛn?

Myelofibrosis yɛ kokoram a emu yɛ den yiye . Mpɛn pii no, ayarefo yi nkwa nna yɛ bɛyɛ mfe asia. Nnipa dodow a wonya nkwa no kyerɛ sɛ nnipa binom ntra ase nnu mfe asia, bere a dodow a ɛte saa ara tra ase bɛboro mfe asia. Eyi nyɛ ade koro mma obiara, ɛsono obiara.

Nneɛma pii wɔ hɔ a ɛkyerɛ sɛnea wo yare no bɛba:

• wo mfe a woadi.
• Wo sɛnkyerɛnne ahorow.
• Wo mogya mu nkwammoaa dodow.
• Sɛnea akuru a ɛwɔ nnompe mu no mu yɛ den.
• Sɛ́ ebia awosu mu nsakrae wɔ hɔ anaasɛ enni hɔ (JAK2, CALR, MPL).

Ade a ɛho hia sen biara ne sɛ wo ne wo duruyɛfo bɛkasa pefee afa sɛnea wo yare no bɛka w’asetra ho.

Mɛyɛ dɛn ahwɛ me ho? (Ne ho a wɔhwɛ) .

Bisa wo duruyɛfo sɛ ebia wubetumi anya ayaresa a ɛma wo ho dwo wo so mfaso anaa . Ebia nnuruyɛfo, ayarehwɛfo, asetra mu nsɛm ho adwumayɛfo, ne animdefo afoforo bɛka akuw a wɔhwɛ wɔn a wɔde wɔn ho to wɔn so no ho. Wobetumi ama wo nneɛma ne mmoa a wuhia na wode atra ase ne yare yi. Wɔ ayaresa a wo nsa ka fi wo kokoram ho ɔyaresafo hɔ akyi no, saa nkurɔfo yi betumi ayɛ mmoa kɛse. Wɔn a wɔyɛ aduruyɛ a ɛma wo ho dwo no betumi aboa ma w’asetra atu mpɔn sɛ obi a ɔwɔ kokoram.

Nea etwa to no, nneɛma a ɛho hia sen biara a ɛsɛ sɛ wokae

Myelofibrosis yɛ mogya mu kokoram a ɛntaa nsi a ntini a ɛyɛ nhama si wo nnompe mu ntini ananmu. Eyi yɛ tebea a emu yɛ den a ɛhwehwɛ sɛ wɔkɔ so hwɛ aduruyɛ so na/anaasɛ wɔsa no yare. Ɛdenam wo tebea no so no, ebia worennya yare no ho sɛnkyerɛnne biara mfe pii. Wɔ tebea afoforo mu no, yare no ho sɛnkyerɛnne betumi akɔ so ntɛmntɛm na ama ayɛ den sɛ wɔbɛyɛ da biara da dwumadi ahorow. Nanso, ayaresa ahorow bi wɔ hɔ a ɛbɛboa ma woadi sɛnkyerɛnne ahorow a ɛhaw wo da biara da asetra no so.

Saa bere yi de, bisa wo duruyɛfo no nneɛma a ebetumi aboa wo ma woagyina nsakrae yi ano ho asɛm. Ɔhwɛ a ɛma obi ho dwo ne akuw a wɔboa no yɛ akwan a ɛboa kɛse bere a woreyɛ nsakrae wɔ asetra a wowɔ kokoram mu no.

Kae sɛ ɛnyɛ wo nko ara. Nnuruyɛfo, abusuafo, ne nnamfo nyinaa wɔ hɔ sɛ wɔbɛboa wo. Nsuro sɛ wo ne wo duruyɛfo bɛkasa afa nsɛmmisa biara a ebia wowɔ ho.

` Myelofibrosis, mogya mu kokoram, nnompe mu ntini, mogya a ɛtɔ kɔ nipadua mu, akyi berɛmo a ɛtrɛw, JAK inhibitors, leukemia