Ukuphononongwa kweeNkcaso zonyango olongezelelweyo
Ulwaphulo oluphela lonyango lwe- myelofibrosis eliphambili (PMF) lugxininisa ukutshintshwa kweeseli , nangona kunjalo, olu cetywayo lunconywa ngabagulane abasemgangathweni nabangaphakathi. Kwale qela, ubudala kunye nezinye iimeko zonyango zinokwandisa ingozi ehambelana nokufakelwa kwezityalo ngokubangela ukuba ibe yinto engaphantsi kwengqondo yonyango. Ukongezelela, akubona bonke abantu abane-PMF enobungozi obuphezulu kunye nabangaphakathi abaya kuba ne-stem efanelekileyo yokutshintshela i-stem (ehambelana nomntakwabo okanye ohambelana nomxhasi ongahambelaniyo).
Kunconywa ukuba abantu abane-PMF enobungozi obuphantsi bafumana unyango olujoliswe ekunciphiseni iimpawu ezihambelana nesi sifo.
Mhlawumbi ugqirha wakho ucebise ukuba ukufakelwa kwezityalo kungeyona nto efanelekileyo kuwe, okanye akukho mnikeli ofanelekileyo onokuqatshelwa, okanye awuzange ubekezelele ezinye iindlela zokuqala zonyango ze-PMF. Ngokwemvelo, umbuzo wakho olandelayo unokuba yiyiphi enye indlela yokwelapha ekhoyo? Ngenhlanhla, kukho ezininzi izifundo eziqhubekayo ezizama ukufumana iinketho ezongezelelweyo zonyango. Siza kuhlolisisa ezinye zala mayeza ngokufutshane.
JAK2 Inhibitors
I-Ruxolitinib , i-JAK2 inhibitor, yayiyonyango lokuqala elijoliswe ku-PMF. Ukuguqulwa kwegazi kuJAK2 kudibaniswe nokuphuhliswa kwe-PMF.
I-Ruxolitinib yonyango olufanelekileyo kubantu abanolu tshintsho olungenako ukutshintshwa kwe-cell stem. Ngethamsanqa, kufunyenwe luncedo nakubantu abangenazo iinguqulelo ze-JAK2.
Kukho uphando oluqhubekayo olubhekiselele ekuphuhliseni amayeza afanayo (ezinye i-JAK2 inhibitors) ezingasetyenziselwa unyango lwe-PMF kunye nokudibanisa i-ruxolitinib nezinye iyeza.
I-Momelotinib yenye enye i-JAK2 inhibitor ifundelwa unyango lwe-PMF. Izifundo zakuqala zathi ama-45 ekhulwini abantu abafumana i-momelotinib banciphise ubukhulu bepen.
Phantse isiqingatha sabantu abafundayo babephuculwe kwi-anemia yabo kwaye abangaphezu kwama-50 ekhulwini babekwazi ukuyeka unyango lwegazi. I-Thrombocytopenia (isibalo esiphantsi seplatelet) sinokuphuhlisa kwaye sinokunciphisa ukusebenza. I-Momelotinib iya kuqhathaniswa ne-ruxolitinib kwisigaba se-3 ukufumana inxaxheba yayo kunyango lwe-PMF.
Izidakamizwa
I-Pomalidomide i-immunomodulatory drug (imishanguzo eguqula isistim somzimba). Inxulumene ne-thalidomide kunye ne-lenalidomide. Ngokuqhelekileyo, la mayeza anikwa nge-prednisone (iyeza le-steroid).
I-Thalidomide kunye ne-lenalidomide sele sele ifundwe njengendlela yokwenza unyango kwi-PMF. Nangona bobabini babonisa inzuzo, ukusetyenziswa kwabo kudla ngokukhawulelana nemiphumo emibi. I-Pomalidomide yaphuhliswa njengento enobuncwane. Ezinye izigulane ziphuculwe kwi-anemia kodwa akukho mpembelelo yabonwa ngobungakanani bentente. Ngenxa yokuba le nzuzo inqununu, kukho izifundo eziqhubekayo ezijongene nokudibanisa i-polmalidomide kunye nezinye i-agents ezinjenge-ruxolitinib kunyango lwe-PMF.
Epigenetic Iidakamizwa
Iziyobisi ze-epigenetic ziyimichiza echaphazela ukuthetha kwamagciwane athile kunokuba ziguqule ngokwenyama. Elinye iklasi lala mayeza anama-hypomethylating agents, eziza kubandakanya i-azacitidine kunye ne-decitabine.
Ezi zonyango zisetyenziswa ekuphatheni i- myelodysplastic syndrome . Izifundo ezijonge indima ye-azacitidine kunye ne-decitabine zikwinqanaba lokuqala. Elinye imithi i-histone deacetlyase (HDAC) inhibitors njenge-givinostat kunye ne-panobinostat.
Everolimus
I-Everolimus yimizi echazwe njenge-mTOR kinase inhibitor kunye ne-immunosuppressant. Ngu-FDA (Ukutya kunye noLawulo lweeDrug) avunyelwe unyango lweengcingo ezininzi (isifuba, isinal cell cellcincin, i-neuroendocrine tumors, njl) kunye nokuthintela ukugatywa komzimba kubantu abafumene ukufakelwa komzimba (isibindi okanye izintso). I-Everolimus ithathwa ngomlomo.
Iziphumo zakuqala zibonisa ukuba kunokunciphisa iimpawu, ubungakanani bepen, i-anemia, isibalo seplatelet kunye nesibalo samhlophe segazi.
Imetelstat
I-Imetelstat iye yafundiswa kwii-kansa ezininzi kunye ne-myelofibrosis. Ekufundeni kwangaphambili, kuye kwadala ukukhululwa (iimpawu ezifihliweyo kunye neempawu ze-PMH) kwabanye abantu abane-PMF ephakathi okanye ephezulu.
Ukuba awuphendulanga unyango lokuqala, ukubhalisa kwilingo lekliniki kunokukunceda ukuba ufikelele kwiimpawu zonyango. Okwangoku, kukho izilingo ezingaphezu kwama-20 ezilingo zokuhlola iindlela zokonyango kubantu abane-myelofibrosis. Unokuxoxa ngale ndlela kunye nodokotela wakho.
> Imithombo:
> Cervantes F. Ndingayiphatha njani imithifibrosis ephambili. Igazi. 2014; 124: 2635-2642.
> Geyer HL kunye neMesa RA. Ulwaphulo lwe-myeloproliferative neoplasms: nini, yiphi i-agent, kwaye njani? Igazi. 2014: 124: 3529-3537.