I-Polycythemia vera yintlungu apho umongo wethambo wenza amangqamuzana ebomvu egazi, amangqamuzana egazi ezimhlophe kunye neeplatelet, ezinokubangela ukuba ingozi yegazi iphakame.
Ukuba uninzi lweeseli zegazi ezibomvu luphawu olugqwesileyo lweklinikhi ye-polycythemia vera. Ukuguqulwa komzimba kwiJAK2, iprotheyini ebandakanyekayo ekutyikitheni ngaphakathi kweseli, ifunyenwe kubantu abaninzi abanalo mqathango.
Ngubani Ofumana I-Polycythemia Vera?
I-Polycythemia vera iyakwenzeka kunoma yiphina ubudala kodwa iyenzeka emva koko ebomini. Ukuba ukhangeleka kwiminyaka yabo bonke abantu abaneli meko, inani eliphakathi kweminyaka elixilongwa ngalo liza kuba yiminyaka engama-60, kwaye ayisoloko ibonwa kubantu abangaphantsi kweminyaka engama-40. Isiganeko se polycythemia vera sincinane kunamadoda kunabesifazane kwaye liphezulu kunamadoda aneminyaka engama-70 ukuya kwengu-79.
Bangaphi abantu abachaphazelekayo?
Kunzima ukuxelela ngokuqinisekileyo, ekubeni umntu unokufumana le meko ixesha elide kwaye engazi, kodwa uqikelelo malunga nomntu omnye okanye ababini kwi-100,000.
Ngokutsho kwe-Incyte Corporation, i-Wilmington, iDelaware, inkampani esekelwe kwi-biopharmaceutical inkampani ekhethekileyo kwi-oncology, kukho abantu abangaba ngama-25,000 e-United States abahlala ne-polycythemia vera kwaye bajongwa njengento engalawulwayo ngenxa yokuba bayakhuthazeka okanye banganyaniseki kwixesha eliphambili lonyango lweziyobisi, i-hydroxyurea .
I-Incyte ibambisana kunye namaziko angama-200 e-community and medical clinics ukuba aqhube isifundo e-US esibizwa ngokuba yi-REVEAL, esandula ukuqala ukubhalisa izigulane, kwaye kulindeleke ukuba zibandakanye izigulane ezingama-2,000 phantsi kolawulo lugqirha ngokuphathwa okusebenzayo kwesi sifo kwithuba kwiminyaka emithathu.
Ngaba Ingumhlaza okanye Isifo?
I-Polycythemia vera inezinye iimpawu ezifana nomhlaza njengoko kubandakanya ukwahlula okungalawulwayo kweseli esilula kwaye akuphiliswa. Ukwazi oku, ukufunda ukuba wena okanye othandekayo unalo mkhuhlane unokuyixinzelela ngokucacileyo. Nangona kunjalo, yazi ukuba le meko ingalawulwa ngempumelelo kwixesha elide kakhulu.
I-National Cancer Institute ichaza i-polycythemia vera ngolu hlobo lulandelayo: "Isifo apho kuninzi amangqamuzana egazi obomvu kumnxeba wegazi kunye negazi, okwenza igazi likhuphe. Inani leeseli ezimhlophe zegazi kunye neeplatelet zinganokwanda. Iiseli zegazi ezongezelelweyo zingaqokelela kwipeni kwaye zenze ukuba zikhuliswe. Zingabangela iingxaki zegazi kunye nokwenza iifom zefestile kwimithambo yegazi. "
Ngokwe-Leukemia & Lymphoma Society, abantu abane-polycythemia vera basengozini encinci kunabantu abemi ekuphuhliseni i-leukemia ngenxa yesifo kunye / okanye ezithile zonyango ezichongiweyo. Nangona le yimeko engapheliyo engapheliyo, khumbula ukuba inokuthi ilawulwe ngokufanelekileyo ngethuba elide, elide-kwaye alisoloko linciphise ixesha lokuphila. Ukongezelela, iingxaki zingaphathwa kwaye zikhuselwe kunye nokulawulwa kwezonyango.
Ngaba Kuqhubeka?
Ewe, kodwa iinkcukacha malunga nomngcipheko wokuqhubela phambili zisacingwa. Nangona abantu bangenako iimpawu kwiminyaka emininzi, i-polycythemia vera inokukhokelela ekuphuhliseni inani leempawu kunye nempawu, kuquka ukukhathala, utywala, ubusuku busizi, intlungu emathanjeni, umkhuhlane kunye nokulahleka kwesisindo. Phantse iipesenti ezingama-30 ukuya kuma-40 ekhulwini labantu abane-polycythemia vera banesipelo esandisiweyo. Kwamanye abantu, kunokukhokelela kwiimpawu eziphosakeleyo kunye neengxaki zentliziyo. Umthwalo wesi sifo usaqhubeka uphando.
Kuchongwa njani?
Uvavanyo olubizwa ngokuba yinkxalabo ye-hematocrit isetyenziselwa ukuxilonga i-polycythemia vera kunye nokulinganisa impendulo yomntu kunyango.
I-Hematocrit iyinxalenye yeeseli ezibomvu zegazi kwigazi, kwaye ngokuqhelekileyo zinikezelwa njengepesenti okanye ukwanda kwe-hemoglobin egazini.
Kubantu abaphilileyo, i-hematocrit concentration isukela kuma-36 ukuya kuma-46 ekhulwini kuma-women kunye nama-42 ukuya kuma-52 ekhulwini kumadoda. Olunye ulwazi olunokuthi luqokelelwe kwiimvavanyo zegazi luyakunceda ekuxilweni, kubandakanywa nobukho benguqu-ukuguquguquka kwe-JAK2-kwiiseli zegazi. Nangona kungadingeki ukuba kwenziwe ukuxilongwa, abanye abantu banokuba nohlalutyo lomongo lwethambo njengenxalenye yokupasa nokuvavanya kwabo.
Uthathwa Njani?
Ngokwe -Leukemia & Lymphoma Society , i-phlebotomy, okanye ukususwa kwegazi kwi-vein, yinto eqhelekileyo yokuqala yonyango kwizigulane ezininzi. Oku kunokunciphisa i-concentration ye-hematocrit, edla ngokubangela ukuphuculwa kweempawu ezithile ezifana neentloko, ukukhala ezindlebeni, kunye nezoxinzelelo.
Ukusetyenziswa kweziyobisi kunokubandakanya ama-agent anganciphisa iiselom ebomvu okanye i-platelet concentrations-iimveliso ezibizwa ngokuba yi-myelosuppressive agents. I-agent ye-myelosuppressive esetyenziswa ngokuqhelekileyo ye-polycythemia vera yi-hydroxyurea, enikwa ngomlomo. Iziphumo eziqhelekileyo ezichaphazelekayo zibandakanya ukukhwehlela okanye ukuphazamiseka, umkhuhlane okanye ukukhupha, ubuhlungu obungaphantsi okanye ubuhlungu bebala, kunye nokuvuthwa okunzima okanye okunzima.
Mayelana neJakafi yePolycythemia Vera
I-Ruxolitinib (i-Jakafi) yonyango lonyango elisetyenziselwa ukuphatha abantu abane-polycythemia vera esele bathathe i-hydroxyurea kwaye ayizange isebenze kakuhle okanye ayikwazanga ukuyinyamezela. I-Jakafi isetyenziselwa ukunyanga iintlobo ezithile ze- myelofibrosis- ukutshatyalaliswa komnatha wethambo.
Ngokutsho kwe-FDA, uJakafi usebenza ngokuvimbela ama-enzyme ebizwa ngokuthi uJanus Associated Kinase (JAK) 1 no-2 abandakanyekayo ekulawuleni igazi kunye nokusebenza kwe-immunological. Ukuvunyelwa kweziyobisi ukuphatha i-polycythemia vera kuya kunceda ukunciphisa ukuvela kwe-spleen-splenomegaly-kunye nesidingo sokuba i-phlebotomy, inqubo yokususa igazi elingaphezu komzimba.
Iziphumo eziqhelekileyo ezichaphazelekayo kunye nokusetyenziswa kweJakafi kubathathi-nxaxheba kunye ne-polycythemia vera kwizilingo ezifanelekayo zeekliniki zazininzi ze-cell red counts (anemia) kunye nokubala okuphantsi kweplatelet yegazi (thrombocytopenia). Imiphumo echaphazelekayo engahambelani negazi, yayingumngcungcutheko, ukuzitywa, kunye neengqungquthela.