Yu bon mכro de mek fayv tisu dεm bak? Lɛ wi lan bɔt maylofibrosis!

Yu dɔn ɛva yɛri bɔt wan sik we dɛn kɔl myelofibrosis? Na tru tru wan kayn blɔd kansa we nɔ kin bɔku. Fɔ tɔk am simpul wan, na we di bon mɛro , we na di saf ɛn spɔnj pat pan yu bon dɛn, gɛt fayv ska tisu in ples . Na wan kayn lukimiya bak we de te fɔ lɔng tɛm, ɛn i de pan wan grup we dɛn kɔl myeloproliferative disorders. Mayloproliferative disorders na we dɛn de mek tumɔs blɔd sɛl dɛn na di bon mɛro, usay dɛn de mek blɔd sɛl dɛn.

Wetin rili na maylofibrosis?

Lɛ wi luk dis mɔ ditayli. Yu bon mɛro gɛt stem sɛl dɛn we de mek blɔd . Leta dɛn stem sɛl dɛn ya kin bi rɛd blɔd sɛl, wayt blɔd sɛl, ɛn pletlɛt. Bɔt pan pɔsin we gɛt maylofibrosis, wan chenj kin apin na di jɛnɛtik matirial ɔ DNA na wan pan dɛn stem sɛl dɛn ya. Dis kin mek di sɛl bi kansa sɛl we nɔ fayn. afta dat dis sel we nכ de wok fayn de sheb εn pas di mכtεshכn to כl di nyu sεl dεm we i de mek.

As tɛm de go, dɛn nɔmal kansa sɛl dɛn ya kin bɔku bɔku wan. sכm pan dεn sεl dεm ya kin mek di bon mכro inflameshn . Dis inflamɛns na in de mek di ska tisu we a bin dɔn tɔk bɔt fɔs fɔm. So, bikɔs ɔf dis ska tisu ɛn di kansa sɛl dɛn we pasmak, di bon mɛro nɔ kin ebul fɔ mek wɛlbɔdi blɔd sɛl dɛn.

Wetin na di men kayn maylofibrosis?

Tu men kayn maylofibrosis de.

• Praymari maylofibrosis: Dis na di kayn we we de apin fɔ insɛf, we nɔ gɛt ɛni kɔnekshɔn wit ɛni ɔda sik.
• Sεkɔndari maylofibrosis: Dis kin kam wit ɔda blɔd disɔda. fכ egzampl, i kin kכz fכ sik dεm lεk praymar trombosaytosis כ polycythemia vera. Dis sεkכnd tכyp de akכnt fכ bitwin 10% εn 20% pan di sik pipul dεm we dεn dכn no.

Aw kɔmɔn dis sik we dɛn kɔl Myelofibrosis?

Maylofibrosis na rili wan sik we nɔ kin apin so ɔltɛm. Fɔ ɛgzampul, na Amɛrika, dɛn kin ripɔt se dis sik kin apin to lɛk 1.5 pan 100,000 pipul dɛn ɛvri ia. I kin afɛkt ɛnibɔdi, ilɛksɛf i ol. Nɔr difrɛns nɔr de pan di ej, bɔt i kin mɔs bi se dɛn kin no am pan pipul dɛm wae dɔn pas 50. If maylofibrosis kam pan yɔŋ pikin dɛm, dɛn kin no am bifo dɛn ol 3 ia.

Us ifɛkt dɛn maylofibrosis kin gɛt pan yu bɔdi?

We dɛn mek di blɔd sɛl dɛn we nɔmal dis we, difrɛn prɔblɛm dɛn kin apin. Lɛ wi tek wan luk pan wetin dɛn bi:

• Anemia: Dis na wan sik wae nɔr de gɛt inof rεd blɔd sɛl. As yu no, na di rɛd blɔd sɛl dɛn we de kɛr ɔksijɛn go ɔlsay na di bɔdi. So we di rεd sεl dεm nכ de, di bכdi in tisu dεm nכ de gεt inof כksijεn. Dis kin mek yu gɛt sɔm kayn sik lɛk fɔ taya, fɔ wik, ɛn fɔ blo shɔt .

• Trombocytopenia: Dis na sik we di pletlɛt dɛn nɔ bɔku. Plɛtlɛt na di tin we de ɛp fɔ mek blɔd klɔt we pɔsin gɛt injuri. So we yu pletlɛt nɔ bɔku, yu bɔdi kin blɔd ɛn brus mɔ .

• Splenomegaly: Yu splin de kɔntrol di blɔd sɛl dɛn ɛn i de pul di rɛd blɔd sɛl dɛn we dɔn pwɛl na yu bɔdi. So we yu mek tumɔs abnɔmal blɔd sɛl dɛn, yu splin gɛt fɔ wok tranga wan. I kin mek i big. We yu splin big, yu kin fil ful ɔ yu nɔ kin fil fayn na di ɔp lɛft pat na yu bɛlɛ .

• εkstramεdul hεmatopoiesis: Dis na di divεlכpmεnt fכ di sεl dεm we de fכm bכdi ausayd di bon mכro , na כda pat dεm na di bכdi. dis sεl dεm kin fכm na ples dεm lεk di lכng, gεstrointestinal trakt, spεnal kכd, bren, כ limf no dεm. Dɛn sɛl ya kin gɛda togɛda fɔ mek tumbu , we kin kam insay ɔda pat dɛn ɔ ambɔg dɛn wok.

• Pɔtal haypatɛnshɔn: Dis na we di blɔd prɛshɔn de go ɔp na di vein we de kɛr blɔd kɔmɔt na yu splin to yu liva. dis kin kכz fכ di damej pan di vein dεm bikoz fכ di εkstramεdul hεmatopoiesis we wi bin dכn tכk bכt.

Wan ɔda tin na dat fɔ lɛk 12% pan di pipul dɛn we gɛt praymari maylofibrosis, di sik kin bi wan kayn blɔd kansa we rili siriɔs we dɛn kɔl akyu mayloid lukimiya .

Wetin kin mek pɔsin gɛt maylofibrosis?

Sayɛnsman dɛn stil nɔ no bɛtɛ bɛtɛ wan wetin kin mek pɔsin gɛt maylofibrosis. Bɔt dɛn dɔn kam fɔ no se i gɛt sɔntin fɔ du wit di chenj dɛn we de apin na di DNA fɔ sɔm jin dɛn . wan kayn protin we dεn kכ l Janus-associated kinases (JAKs) de involv. dis JAKs protin dεm de kכntro di prodyushכn fכ di bכdi sεl dεm na di bon mכro, we de sεnd signal to di sεl dεm fכ divayd εn gro. if dεn JAKs protin dεm ya de wok pasmak, tu mכch כ tu sכm bכdi sεl dεm kin kכmכt.

bitwin 60% εn 65% pan di pipul dεm we gεt maylofibrosis gεt mכtεshכn na di JAK2 jin . Wan ɔda 5% to 10% .wan mכtεshכn de na di myeloproliferative leukemia (MPL) jin. כlso, dεn kin si wan mכtεshכn we dεn kכl Calreticulin (CALR) insay bitwin 20% εn 25% pan di wan dεm we gεt maylofibrosis.

Udat de pan denja pas ɔl fɔ dis?

Yu kin gɛt mɔ risk fɔ gɛt maylofibrosis fɔ dɛn rizin ya:

• If yu dɔn pas 50 ia .
• If yu gɛt blɔd disɔda we dɛn kɔl praymari trombosaytɔsis ɔ polycythemia vera.
• If yu dɔn gɛt ayɔniz raytin ɔ petrokemikal lɛk bɛnzin ɛn toluɛn.

Wetin na di sayn dɛm fɔ maylofibrosis?

Maylofibrosis na sik we de go bifo smɔl smɔl , so yu nɔ go gɛt ɛni sayn fɔ lɔng tɛm. Na lɛk wan pat pan tri pan di sik pipul dɛn nɔ kin gɛt ɛni sayn we dɛn kin bigin.

If di sayn dɛn we de sho se yu gɛt dis sik, di wan dɛn we kin apin mɔ na we yu taya bad bad wan (bikɔs yu nɔ gɛt blɔd) ɛn yu splin kin big . Di sayn dɛm kin bi bak:

• Wok tranga wan
• Fiva
• I de it
• Pale skin
• We yu de lɔs yu wet
• Nayt swet dɛn
• Bon ɔ jɔyn pen
• Infekshɔn dɛn we kin kam bɔku tɛm
• Spleen ɔ liva we dɔn big
• Blɔd we de klɔt we dɛn nɔ ɛksplen
• Blɔd ɔ brus we nɔ kɔmɔn
• di vein dεm we de na di bכdi εn di εsophagus de big (dεn vein dεm ya kin bכs εn blכd).

Aw fɔ no bɔt dis sik?

Dɔktɔ, mɔ wan dɔktɔ we de mɛn kansa, go chɛk yu bɔdi, aks yu bɔt yu mɛrɛsin istri, ɛn aks yu bɔt di sik dɛn we yu gɛt naw. Dɛn go chɛk bak fɔ si if di splin dɔn big ɛn if i gɛt sayn dɛn we de sho se pɔsin nɔ gɛt blɔd.

Dɔn, dɛn kin du difrɛn tɛst dɛn fɔ no ɔda tin dɛn ɛn fɔ no if yu gɛt maylofibrosis.

Blɔd tɛst dɛn

• Kɔmplit blɔd kɔnt (CBC): Dis de sho di nɔmba fɔ difrɛn kayn sɛl dɛn na yu blɔd. If yu rɛd blɔd sɛl dɛn smɔl pas aw i fɔ bi, ɔ yu wayt blɔd sɛl ɛn pletlɛt dɛn nɔ de wok fayn, dat kin bi sayn fɔ se yu gɛt maylofibrosis.
• Peripheral blood smear (PBS): Dis kin chɛk di sayz, shep, ɛn ɔda tin dɛn we yu blɔd sɛl dɛn gɛt fɔ si if dɛn gɛt tin dɛn we nɔ fayn. If sɛl dɛn de we nɔ de luk fayn ɛn bɔku bɔku blɔd sɛl dɛn de we nɔ machɔ, i kin bi sayn bak fɔ se pɔsin gɛt maylofibrosis.
• Blɔd kemistri tɛst dɛn:Dɛn tɛst ya kin no di lɛvɛl we difrɛn tin dɛn we kin kɔmɔt na yu ɔgan dɛn kin kɔmɔt na di blɔd. Dis kin tɛl yu aw wan ɔgan de wok fayn fayn wan. if yu gεt hכy lεvεl fכ tin dεm lεk yurik asid, bilirubin, εn laktik dihaydrojenεz na di bכdi kin bi sayn bak fכ maylofibrosis.

Tɛst fɔ di bon mɛro

• Bone marrow biopsy: Dis na fɔ tek smɔl sampul pan yu bon marrow ɛn chɛk am ɔnda maykroskɔp. dεn de analכz di sεl dεm fכ no if yu gεt maylofibrosis.
• di bon mכro aspirashכn: Dis involv fכ pul di wata we de kכmכt na di bon mכro εn tεst am fכ sayn dεm fכ maylofibrosis.

Sɔntɛm dɛn go nid fɔ du ɔda tɛst dɛn

Yu kin nid fɔ du ɔda tɛst fɔ no if yu gɛt di sik:

• Jin mכtεshכn analisis: Yu dכkta go tεst yu blכd εn bon mכro sεl dεm fכ jin mכtεshכn we kכnεkt wit maylofibrosis, lεk JAK2, CALR, εn MPL. Sɔm tritmɛnt dɛn kin tɔch di kansa sɛl dɛn we gɛt di JAK2 muteshon.
• Di we aw fɔ tek pikchɔ: Yu dɔktɔ kin du ɔltra saund fɔ chɛk if yu splin dɔn big. Dɛn kin du MRI bak fɔ chɛk fɔ si if ska tisu de na di bon mɛrɔ. Dis ska tisu kin bi sayn fɔ maylofibrosis.

Aw dɛn kin trit maylofibrosis?

If yu nɔr de sho sɔm kayn sik, yu nɔr nid tritmɛnt. Bɔt ivin if yu nɔ nid tritmɛnt kwik kwik wan, yu dɔktɔ go kɔntinyu fɔ wach yu sik.

Jakafi® (ruxolitinib), Inrebic® (fedratinib), ɛn Vonjo® (pacritinib) na mɛrɛsin dɛn we di U.S. Food and Drug Administration (FDA) dɔn gri fɔ . Dɛn kin yuz dɛn fɔ trit mɔdaret to ay risk maylofibrosis. Ɔl dɛn tri mɛrɛsin ya de wok lɛk JAK inhibito . dat min se dεn de ridyus di כva aktv Janus-asכsiet kinaz (JAK) signal. Dis dɔn mek dɛn mɛrɛsin ya kin ɛp fɔ pul di sik dɛn we gɛt fɔ du wit maylofibrosis, lɛk we yu splin big, we yu de swet na nɛt, we yu de it, we yu de lɔs yu wet, ɛn fiva.

Fɔ bɔku pipul dɛn, di men gol fɔ tritmɛnt na fɔ manej di kɔndishɔn dɛn we gɛt fɔ du wit maylofibrosis, lɛk anemia ɛn di splin we big.

Tritmɛnt fɔ Anemia

Tritmɛnt dɛn de fɔ anemia lɛk:

• Androgens: If yu tek androgens lεk danazol i kin mek rεd bכdi sεl dεm prodyuz bכku.
• Immunomodulators: Dɛn mɛrɛsin ya kin mek yu imyun sistɛm ebul fɔ fɛt di kansa sɛl dɛn, ɛn dis kin mek di sik nɔ bɔku. Drug dεm lεk intafεron, thalidomide (Thalomid®), εn lenalidomide (Revlimid®) de pan dis klas. Dɛn kin gi dɛn bak togɛda wit glukokortikoyd dɛn lɛk prɛdnisɔn.
• Kimotɛrapi drɔgs: Sɔm kemotɛrapi drɔgs kin ridyus di sayn dɛm we kin kam bikɔs di blɔd sɛl dɛn kin bɔku ɛn di splin kin big. Hydroxyurea ɛn cladribine na dɛn kayn mɛrɛsin ya.
• Blɔd transfyushɔn: If yu gɛt siriɔs anemia, if yu de gi yu blɔd ɔltɛm, dat kin mek yu rɛd blɔd sɛl dɛn bɔku.

Tritmɛnt fɔ Splenomegaly

Dɛn kin yuz JAK inhibito, immunomodulator, ɛn kemotɛrapi drɔgs fɔ manej wan splin we dɔn big. If i siriɔs, yu kin nid fɔ mek dɛn du ɔpreshɔn fɔ pul yu splin (splenectomy) ɔ gɛt raytin tɛrapi to di splin.

rεdyushכn tεrapi kin nid bak fכ trit wan kכndyushכn we dεn kכl εkstramεdula hεmatopoiesis, we di bכdi sεl dεm de divεlכp ausayd di bon mכro.

Yu tink se dɛn kin mɛn maylofibrosis kpatakpata?

Allogeneic hematopoietic cell transplantation (HCT) kin bi di onli opshɔn fɔ mɛn dis sik. Bɔt dis na tin we kin mek pɔsin gɛt prɔblɛm ɛn i nɔ fayn fɔ ɔlman.

Insay dis we aw dɛn kin du dis, dɛn kin chenj yu blɔd sɛl dɛn we nɔ nɔmal, wit sɛl dɛn we kɔmɔt frɔm pɔsin we gɛt wɛlbɔdi. Bifo dɛn transplant yu, dɛn go gi yu kemotɛrapi ɔ redyushɔn tɛrapi fɔ kil yu sɛl dɛn we gɛt sik. Dɔn, di nyu sɛl dɛn frɔm di pɔsin we gi yu kin tek di wok dɛn we yu bɔdi de du.

HCT tritmɛnt kin gɛt ay risk fɔ gɛt kɔmplikeshɔn dɛn . So, i fayn fɔ sɔm pipul dɛn nɔmɔ we dɛn dɔn pik. De risk fɔ gɛt kɔmplikeshɔn kin ivin pasmak fɔ pipul dɛm wae gɛt ɔda mɛrɛsin. If yu fit fɔ dis, i kin dipen pan bɔku tin, lɛk yu ej, aw yu sik bad, ɛn aw di tritmɛnt go ebul fɔ du am fayn.

Aw de layf kin tan lɛk wae yu de liv wit dis sik?

Maylofibrosis na wan kansa we rili siriɔs . di midyan rεt fכ dεn sik pipul ya kin bi lεk siks ia. Di midyan sɔvayv rit min se sɔm pipul dɛn nɔr de liv lɛk siks ia, ɛn di sem nɔmba de liv fɔ pas siks ia. Dis nɔr kin bi di sem fɔ ɔlman, i kin difrɛn frɔm wan pɔrsin to ɔda pɔrsin.

Bɔrku tin de wae de sho aw yu go gɛt yu sik:

• yu ej.
• Yu simptom dɛm.
• Yu blɔd sɛl dɛn kɔnt.
• Di kayn we aw skata kin rili bad na di bon mɛrɔ.
• if jεnεtik mכtεshכn de כ nכ de (JAK2, CALR, MPL).

De tin wae impɔtant pas ɔl na fɔ tɔk to yu dɔktɔ opin wan bɔt aw di sik wae yu gɛt go afɛkt yu layf.

Aw a kin kia fɔ misɛf? (Fɔ kia fɔ yusɛf) .

Aks yu dɔktɔ if yu go bɛnifit frɔm palliativ kia . Di tim dɛn we de kia fɔ pipul dɛn we nɔ gɛt sik kin gɛt dɔktɔ dɛn, nɔs dɛn, pipul dɛn we de wok fɔ pipul dɛn, ɛn ɔda spɛshal pipul dɛn. Dɛn kin gi yu di tin dɛm ɛn sɔpɔt wae yu nid fɔ liv wit dis sik. Apat frɔm di tritmɛnt we yu kin gɛt frɔm yu dɔktɔ we de mɛn yu kansa, dɛn pipul ya kin bi big sɔpɔt fɔ yu. Palliative care specialist kin ɛp fɔ impɔtant yu kwaliti fɔ liv as pɔrsin wae de liv wit kansa.

Fɔ dɔn, di tin dɛn we impɔtant pas ɔl we yu fɔ mɛmba

Maylofibrosis na wan kayn blɔd kansa we nɔ kin apin so ɔltɛm we yu bon mɛrɔ kin tek di ples wit fayv tisu. Dis na siriɔs kɔndishɔn we nid fɔ kɔntinyu fɔ de wach ɛn/ɔ tritmɛnt. Dipen pan yu sik, yu nɔr kin gɛt ɛni sayn fɔ lɔng tɛm. Ɔda tɛm, di sayn dɛm kin go bifo kwik kwik wan ɛn kin mek i nɔr izi fɔ du tin dɛm ɛvride. Bɔt sɔm tritmɛnt dɛn de fɔ ɛp fɔ kɔntrol di sik dɛn we de ambɔg yu ɛvride layf.

Insay da tɛm de, aks yu dɔktɔ bɔt tin dɛn we go ɛp yu fɔ bia wit dɛn chenj ya. Palliative care ɛn sɔpɔt grup na opshɔn wae kin rili ɛp yu as yu de ajɔst to layf wit kansa.

Mɛmba se nɔto yu wangren de. Dɔktɔ dɛn, fambul dɛn, ɛn padi dɛn ɔl de fɔ ɛp yu. Nɔ fred fɔ tɔk to yu dɔktɔ bɔt ɛni kwɛstyɔn we yu go gɛt.

` Maylofibrosis, blɔd kansa, bon mɛro, anemia, splin big, JAK inhibito, lukimia