Yintoni i-Myelofibrosis yaseprayimari?

Umxholo

• Iimpawu zokuqala ze-myelofibrosis
• Amanqanaba okuqala e-myelofibrosis
• Yintoni ebangela i-myelofibrosis ephambili?
• Imiba yomngcipheko kwi-myelofibrosis ephambili
• Unyango lokuqala lwe-myelofibrosis
• Amayeza okulawula iimpawu
• I-JAK inhibitors
• Ukutshintshwa kweseli
• I-Chemotherapy kunye ne-radiation
• Utofelo-gazi
• Ugqirha
• Uvavanyo lweklinikhi lwangoku
• Utshintsho kwindlela yokuphila
• Imbonakalo
• Yise kude

I-myelofibrosis yaseprayimari (MF) ngumhlaza onqabileyo obangela ukwakheka kwezicubu ezibomvu, ezaziwa ngokuba yi-fibrosis, kumongo wethambo. Oku kuthintela umongo wethambo lakho ekuveliseni isiqhelo seeseli zegazi.

I-Primary MF luhlobo lomhlaza wegazi. Ngenye yeentlobo ezintathu ze-myeloproliferative neoplasms (MPN) eyenzeka xa iiseli zahlulahlula rhoqo okanye zingafi rhoqo njengoko kufanele. Ezinye ii-MPNs zibandakanya i-polycythemia vera kunye ne-thrombocythemia ebalulekileyo.

Oogqirha bajonga izinto ezininzi zokuchonga i-MF yokuqala. Unokufumana uvavanyo lwegazi kunye nomongo wethambo ukuze ufumanise i-MF.

Iimpawu zokuqala ze-myelofibrosis

Awunakufumana naziphi na iimpawu iminyaka emininzi. Iimpawu zihlala ziqala ukwenzeka ngokuthe ngcembe emva kokuqhekeka komongo wethambo kwaye ziqala ukuphazamisa ukuveliswa kweeseli zegazi.

Iimpawu zokuqala ze-myelofibrosis zingabandakanya:

• ukudinwa
• ukuqhawukelwa ngumphefumlo
• ulusu oluthuthu
• ifiva
• usulelo rhoqo
• ukutyunyuzwa ngokulula
• ukubila ebusuku
• Ukuphelelwa ngumdla wokutya
• ukwehla kobunzima obungachazwanga
• iintsini ezophayo
• ukopha rhoqo
• ukugcwala okanye iintlungu esiswini kwicala lasekhohlo (kubangelwa sisibindi esandisiweyo)
• iingxaki nokusebenza kwesibindi
• ukurhawuzelela
• intlungu edibeneyo okanye yethambo
• igawuthi

Abantu abane-MF bahlala benenqanaba eliphantsi kakhulu leeseli ezibomvu zegazi. Banokuba nenani elimhlophe leeseli zegazi eziphakamileyo kakhulu okanye eziphantsi kakhulu. Ugqirha wakho unokufumana kuphela oku kungalunganga ngexesha lokuhlolwa rhoqo kulandela ubalo olupheleleyo lwegazi.

Amanqanaba okuqala e-myelofibrosis

Ngokungafaniyo nezinye iintlobo zomhlaza, i-MF yaseprayimari ayinazo izigaba ezichazwe ngokucacileyo. Ugqirha wakho endaweni yoko unokusebenzisa inkqubo yeDynamic International Prognostic Scoring System (DIPSS) ukukuhlela ngokweqela eliphantsi, eliphakathi-, okanye elinomngcipheko omkhulu.

Baza kujonga ukuba ngaba:

• banenqanaba lehemoglobin elingaphantsi kwe-10 gram kwidesilitha nganye
• Unobalo lweeseli ezimhlophe zegazi ezikhulu kune-25 × 10⁹ ilitha nganye
• badala kwiminyaka engama-65 ubudala
• ukujikeleza kweeseli ezilinganayo okanye ezingaphantsi kwepesenti enye
• amava iimpawu ezifana nokudinwa, ukubila ebusuku, umkhuhlane kunye nokwehla kobunzima

Uthathwa njengomngcipheko ophantsi ukuba akukho nanye kwezi ingasentla isebenza kuwe. Ukudibana nenye okanye ezimbini zezi ndlela kukubeka kwiqela eliphakathi lomngcipheko. Ukudibana ezintathu okanye nangaphezulu kwezi ndlela kukubeka kwiqela elinomngcipheko omkhulu.

Yintoni ebangela i-myelofibrosis ephambili?

Abaphandi abayiqondi kakuhle into ebangela i-MF. Idla ngokungafunyanwa njengelifa ngokwemfuza. Oko kuthetha ukuba awukwazi ukufumana esi sifo kubazali bakho kwaye awukwazi ukusidlulisela kubantwana bakho, nangona i-MF ithanda ukuqhuba kwiintsapho. Olunye uphando lubonisa ukuba kunokubangelwa kukutshintsha kwemfuza okuchaphazela iindlela zokubonisa iiseli.

Abantu abane-MF banokutshintsha kwemfuza okubizwa ngokuba yi-janus-associated kinase 2 (IJAK2echaphazela iiseli zeziqu zegazi. Inkqubo ye- IJAK2 Ukutshintsha komzimba kudala ingxaki kwindlela umongo wethambo ovelisa ngayo iiseli ezibomvu zegazi.

Iiseli zegazi ezingaqhelekanga kumongo wethambo zenza iiseli zegazi ezivuthiweyo eziphindaphindayo ngokukhawuleza kwaye zithathe umongo wethambo. Iseli yegazi eyandayo ibangela amanxeba kunye nokudumba okuchaphazela amandla omongo wethambo ukwenza iiseli eziqhelekileyo zegazi. Oku kuhlala kukhokelela kwizimbalwa kuneeseli ezibomvu zegazi kunye neeseli ezimhlophe zegazi ezininzi kakhulu.

Abaphandi banxibelelanise i-MF nolunye uguquko. Phantse iipesenti ezi-5 ukuya kwezi-10 zabantu abane-MF bane- MPL Ukutshintsha kwemfuza. Malunga neepesenti ezingama-23.5 zinokutshintshwa kwemfuza okubizwa ngokuba yi-calreticulin (ICALR).

Imiba yomngcipheko kwi-myelofibrosis ephambili

I-Primary MF inqabile. Kwenzeka kuphela malunga ne-1.5 kubantu abangama-100,000 e-United States. Esi sifo sinokuchaphazela amadoda nabasetyhini.

Imiba embalwa inokunyusa umngcipheko womntu wokufumana i-MF ephambili, kubandakanya:

• ungaphezulu kweminyaka engama-60 ubudala
• ukubonakaliswa kwimichiza ye-petrochemicals efana ne-benzene kunye ne-toluene
• ukubonakaliswa kwimitha ye-ionizing
• ukuba ne IJAK2 Ukutshintsha kwemfuza

Unyango lokuqala lwe-myelofibrosis

Ukuba awunazo iimpawu ze-MF, ugqirha wakho usenokungakubeki kulo naluphi na unyango kwaye endaweni yoko akubeke esweni ngononophelo ngokujonga okuqhelekileyo. Nje ukuba iimpawu ziqale, unyango lujolise ekulawuleni iimpawu kunye nokuphucula umgangatho wobomi.

Unyango lokuqala lwe-myelofibrosis lonyango lubandakanya amayeza, i-chemotherapy, i-radiation, ukufakelwa kweeseli, ukutofelwa igazi kunye nokuhlinzwa.

Amayeza okulawula iimpawu

Amayeza aliqela anokunceda ekuphatheni iimpawu ezinjengokudinwa kunye nokunqanda igazi.

Ugqirha wakho unokuncoma i-aspirin yedosi ephantsi okanye i-hydroxyurea ukunciphisa umngcipheko we-venous thrombosis (DVT).

Amachiza okuphatha ukubala kweeseli ezibomvu zegazi (i-anemia) enxulunyaniswe neMF ibandakanya:

• Unyango lwe-androgen
• i-steroids, njenge-prednisone
• thalidomide (Thalomid)
• lenalidomide (ihlaziyiweyo)
• iiarhente ezikhuthazayo ze-erythropoiesis (ii-ESA)

I-JAK inhibitors

I-JAK inhibitors ziphatha iimpawu ze-MF ngokuthintela umsebenzi we IJAK2 gene kunye neprotein yeJAK1. I-Ruxolitinib (Jakafi) kunye ne-fedratinib (i-Inrebic) ngamayeza amabini avunyiweyo yi-Food and Drug Administration (i-FDA) ukunyanga umngcipheko ophakathi okanye umngcipheko omkhulu we-MF. Ezinye ii-inhibitors ze-JAK ziyavavanywa kuvavanyo lweklinikhi.

I-Ruxolitinib ibonakalisiwe ukunciphisa ukwandiswa kwepleen kunye nokunciphisa uninzi lweempawu ezinxulumene neMF, ezinje ngokuqaqanjelwa sisisu, iintlungu zethambo kunye nokurhawuzelela. Iyanciphisa amanqanaba e-cytokines esephusheni egazini. Oku kunokunceda ukunciphisa iimpawu ze-MF kubandakanya ukudinwa, umkhuhlane, ukubila ebusuku, kunye nokwehla kobunzima.

I-Fedratinib ihlala inikwa xa i-ruxolitinib ingasebenzi. Yeyona JAK2 inamandla yokukhetha inhibitor. Inomngcipheko omncinci wokulimala kwengqondo kunye nobungozi obunokubulala obubizwa ngokuba yi-encephalopathy.

Ukutshintshwa kweseli

Ukufakelwa kwe-cell allogeneic stem (ASCT) kuphela kwonyango olunokubakho lwe-MF. Eyaziwa nangokuthi kukutsalwa komongo wethambo, kubandakanya ukwamkelwa kokufakwa kweeseli ezinamanzi kumxhasi osempilweni. Ezi seli ze-stem zisempilweni zithatha indawo yeeseli ezingasebenzi kakuhle.

Inkqubo inomngcipheko omkhulu wobungozi obusongela ubomi. Uya kuhlolisiswa ngononophelo ngaphambi kokuba utshatise umnikeli. I-ASCT iqwalaselwa kuphela kubantu abanomngcipheko ophakathi okanye umngcipheko ophezulu kwi-MF abangaphantsi kweminyaka engama-70.

I-Chemotherapy kunye ne-radiation

Amachiza e-Chemotherapy kubandakanya i-hydroxyurea inokunceda ukunciphisa ipeni eyandisiweyo enxulunyaniswe neMF. Unyango ngemitha ngamanye amaxesha luyasetyenziswa xa i-JAK inhibitors kunye ne-chemotherapy azonelanga ukunciphisa ubungakanani bepeni.

Utofelo-gazi

Ukufakwa kwegazi iiseli ezibomvu ezisempilweni kunokusetyenziselwa ukwandisa ukubala kweeseli ezibomvu nokunyanga i-anemia.

Ugqirha

Ukuba i-spleen eyandisiweyo ibangela iimpawu ezinzima, ugqirha wakho ngamanye amaxesha unokucebisa ukususwa kotyando. Le nkqubo yaziwa ngokuba yi-splenectomy.

Uvavanyo lweklinikhi lwangoku

Amaninzi amachiza aphantsi kophando kunyango lwe-myelofibrosis. Oku kubandakanya ezinye iziyobisi ezininzi ezithintela i-JAK2.

Isiseko soPhando se-MPN sigcina uluhlu lwezilingo zeklinikhi ze-MF. Ezinye zezi zilingo sele ziqalisile ukuvavanya. Abanye ngoku bathatha izigulana. Isigqibo sokujoyina uvavanyo lwezonyango kufuneka senziwe ngononophelo nogqirha kunye nosapho.

Iziyobisi zihamba ngezigaba ezine zovavanyo lweklinikhi ngaphambi kokufumana imvume yi-FDA. Ambalwa kuphela amachiza amatsha okwangoku akwinqanaba lesigaba sesithathu sezilingo zeklinikhi, kubandakanya i-pacritinib kunye ne-momelotinib.

Uvavanyo lweSigaba I kunye no-II lwezonyango lubonisa ukuba i-everolimus (RAD001) inokunceda ukunciphisa iimpawu kunye nobungakanani bepeni kubantu abane-MF. Eli chiza lithintela indlela kwiiseli ezivelisa igazi ezinokuthi zikhokelele ekukhuleni kweseli okungaqhelekanga kwi-MF.

Utshintsho kwindlela yokuphila

Unokuziva uxinezelekile ngokwasemphefumlweni emva kokufumana isifo sokuqala se-MF, nokuba awunazo iimpawu. Kubalulekile ukucela inkxaso kusapho nakubahlobo.

Ukudibana nomongikazi okanye unontlalontle unokukunika ulwazi oluninzi malunga nokuba isifo somhlaza singabuchaphazela njani ubomi bakho. Unokufuna ugqirha wakho malunga nokusebenza nengcali yezempilo yengqondo enelayisensi.

Olunye utshintsho kwindlela yokuphila lunokukunceda ulawule uxinzelelo. Ukucamngca, iyoga, ukuhamba kwendalo, okanye ukumamela umculo kunokunceda ukukhulisa imo yakho kunye nokuba sempilweni ngokupheleleyo.

Imbonakalo

I-MF yaseprayimari ayinakubangela iimpawu kumanqanaba ayo okuqala kwaye inokulawulwa ngeendlela ezahlukeneyo zonyango. Ukuqikelela imbonakalo kunye nokusinda kwe-MF kunokuba nzima. Esi sifo asiqhubeki ixesha elide kwabanye abantu.

Uqikelelo lokusinda luhlu kuxhomekeke ekubeni umntu ukwinqanaba eliphantsi, eliphakathi, okanye elinomngcipheko omkhulu. Olunye uphando lubonisa ukuba abo bakwiqela elinomngcipheko ophantsi banamazinga afanayo okusinda kwiminyaka emihlanu yokuqala emva kokuchongwa njengabantu ngokubanzi, apho amaqondo okusinda aqala ukwehla. Abantu abakwiqela elinomngcipheko omkhulu basindile ukuya kuthi ga kwiminyaka esi-7.

I-MF inokukhokelela kwiingxaki ezinkulu ekuhambeni kwexesha. I-MF yaseprayimari iqhubela phambili kwisifo somhlaza esibi kakhulu nesinzima ukunyanga esaziwa ngokuba yi-acute myeloid leukemia (AML) malunga neepesenti ezili-15 ukuya kwezingama-20 zamatyala.

Uninzi lonyango lwe-MF ephambili lujolise kulawulo lweengxaki ezinxulunyaniswe neMF. Oku kubandakanya i-anemia, i-spleen eyandisiweyo, iingxaki zokunqanda igazi, ukuba neeseli ezimhlophe zegazi okanye iiplatelets, kunye nokubala okuphantsi kweplatelet. Unyango lukwalawula iimpawu ezinjengokudinwa, ukubila ebusuku, isikhumba esirhawuzelelayo, umkhuhlane, iintlungu ezidibeneyo, kunye negawuthi.

Yise kude

I-Primary MF ngumhlaza onqabileyo ochaphazela iiseli zakho zegazi. Abantu abaninzi abayi kuba neempawu ekuqaleni de umhlaza uqhubeke. Olona nyango lunokubakho kunyango lwe-MF olusisiseko kukufakelwa kweseli, kodwa kukho ezinye iindlela zonyango kunye nezilingo zeklinikhi eziqhubekayo ukulawula iimpawu kunye nokuphucula umgangatho wobomi.