Polycythemia vera chirwere umo mafuta emuti anoita masero akawanda matsvuku eropa, masero eropa, uye maplatelet, izvo zvinogona kutungamirira kuwedzerwa ngozi yekuvhara ropa.
Kuva nemasero matsvuku emuropa ndiyo inonyanya kuratidzira chiratidzo chekliniki ye polycythemia vera. Kushandurwa kwegene reJAK2, puroteni yakabatanidzwa mukuisa chiratidzo mukati memusero, yakawanikwa kune vanhu vazhinji vane chirwere ichi.
Ndiani Anowana Polycythemia Vera?
Polycythemia vera inogona kuitika chero ipi zvayo asi inowanzoitika gare gare muupenyu. Kana iwe uchitarisa makore evanhu vose vane chirwere ichi, nhamba yepakati yemakore ekuongororwa ichave makore makumi matanhatu, uye haiwanzoonekwi kune vanhu vari pasi pemakore makumi mana. Chiitiko che polycythemia vera chiri nani kupfuura varume uye yakakwirira kune varume vane makore makumi manomwe kusvika ku79.
Vanhu Vakawanda Vanobatwa Sei?
Zvakaoma kutaura zvakanaka, sezvo munhu angave ane chirwere ichi kwenguva yakareba uye asingazvizivi, asi kuongororwa kune imwechete kana vanhu vaviri kubva kune 100 000.
Maererano neIncyte Corporation, Wilmington, Delaware, yakagadzirwa nekambani ye biopharmaceutical inonyanya kuongorora incology, kune vanhu vanenge 25 000 vari muUnited States vanogara ne polycythemia vera uye vanoonekwa sevasingadzorwi nokuti vanodzivisa kusagadzikana kana kusagamuchirwa kwechirwere chemishonga inonzi therapy, hydroxyurea .
Incyte inobatanidzwa pamwe nemasangano makumi maviri nemazana emasangano ezvokurapa kuti adzidze muUnited States inonzi REVEAL, iyo ichangobva kutanga kunyoresa varwere, uye inotarisirwa kuisa varwere 2 000 pasi pekutarisirwa kwachiremba wekushanda kwechirwere ichi kwenguva wemakore matatu.
Icho Chirwere Here Kana Kuti Chirwere?
Polycythemia vera ine mamwe maitiro akafanana nekenza sezvo inosanganisira kusadzora kusadzorwa kwechinhu chisina kuchena uye hachisi kupora. Kuziva izvi, kudzidza kuti iwe kana munhu waunoda ane chirwere ichi chinonzwisisika chinoshungurudza. Zvisinei, ziva kuti izvi zvinogona kutarisirwa zvakanaka kwenguva refu.
Bhuku reNational Cancer Institute rinotsanangura polycythemia vera sezvinotevera: "Chirwere umo mune masero masero matsvuku eropa mumapfupa eropa neropa, zvichiita kuti ropa rive. Nhamba yemasero machena eropa uye maplatelet inogonawo kuwedzera. Mamwe masero eropa anokwanisa kuunganidzirwa muchero uye anoita kuti iwedzere. Zvinogona kukonzera zvinetso zvekubuda ropa uye kuita mavhatomu mafomu mumidziyo yeropa. "
Maererano neLeukemia & Lymphoma Society, vanhu vane polycythemia vera vari pangozi duku kudarika huwandu hwevanhu hwekukura kwehuukemukari nekuda kwechirwere uye / kana mamwe maitiro ekurapa kwemishonga. Kunyangwe ichi chiri chimiro chisingaperi chisingarapiki, yeuka kuti chinogona kutariswa zvakanaka kwenguva refu, kwenguva yakareba-uye haisi kupfupisa nguva yeupenyu. Uyezve, zvinetso zvinogona kurapwa uye kudzivirirwa neutano hwezvechiremba.
Inofambira Mberi Here?
Hungu, asi mashoko pamusoro pengozi yekufambira mberi achiri kuongororwa. Kunyange zvazvo vanhu vangave vasina zviratidzo kwemakore akawanda, polycythemia vera inogona kutungamirira kukurudzirwa kwezviratidzo nezviratidzo zvakasiyana, kusanganisira kuneta, utchiness, usiku sweats, kurwadziwa mumapfupa, fivha uye kuora kurasikirwa. Inenge 30 muzana kusvika 40 muzana yevanhu vane polycythemia vera vane chirwere chakawedzerwa. Mune vamwe vanhu, zvinogona kutungamirira ku zviratidzo zvinoparadza uye zvinetso zvemoyo. Chiremera chechirwere ichi chiri chichiri kunyatsotsvakurudzwa.
Inozivikanwa Sei?
Muedzo unonzi hematocrit concentration inoshandiswa zvose kuti uongorore polycythemia vera uye kuongorora mhinduro yomunhu kune kurapa.
Hematocrit ndiyo chikamu chemasero matsvuku eropa muropa reropa, uye rinowanzopiwa seperesenti kana kuwedzerwa kwehemoglobin yekufungidzirwa muropa.
Muvanhu vane utano, hematocrit concentration inenge inenge 36 kusvika 46 muzana yevakadzi uye 42 kusvika ku 52 muzana mumarume. Dzimwe ruzivo runogona kuwanikwa kubva kuongororo yeropa dzinobatsirawo mukuongororwa, kusanganisira kuvapo kwekuchinja-kweJAK2 mutation-mune masero eropa. Kunyange zvazvo zvisingadi kuti zviongororwe, vamwe vanhu vanogonawo kunge vakave nokuongorora marora sechikamu chekubata kwavo nekuongorora.
Inobatwa Sei?
Maererano neLeukemia & Lymphoma Society , phlebotomy, kana kubviswa kweropa kubva mumvura, ndiyo inowanzotanga mapiritsi ekurapa kwevakawanda varwere. Izvi zvinogona kuderedza humatocrit concentration, iyo inowanzokonzera kugadziridzwa kwemamwe zviratidzo zvakadai semusoro, kurira munzeve, uye chizuru.
Kushandisa zvinodhaka kunogona kusanganisira mashizha anokwanisa kuderedza tsvuku tsvuku kana platelet kuwedzera-makemikari anonzi myelosuppressive agents. Myelosuppressive agent inonyanya kushandiswa ye polycythemia vera i hydroxyurea, inopiwa nemuromo. Zvimwe zvinowanzova nemigumisiro zvinosanganisira kuora kana kushaya simba, fever kana chills, kuchera kumashure kana kurwadziwa kwepamuviri, uye kunorwadza kana kuoma kuoma.
Pamusoro peJakafi yePolythemia Vera
Ruxolitinib (Jakafi) mishonga yemishonga inoshandiswa kurapa vanhu vane polycythemia vera avo vatotora hydroxyurea uye haina kushanda zvakanaka zvakakwana kana kuti havagoni kuitakura. Jakafi inoshandiswawo kubata mamwe maitiro e- myelofibrosis -kuora kwefupa remafu.
Maererano neDFA, Jakafi inoshanda nekudzivisa maitiro enyama anonzi Janus Associated Kinase (JAK) 1 uye 2 ayo anobatanidzwa mukurapa ropa uye kushanda kweunofuniti. Mvumo yezvinodhaka yekurapa polycythemia vera ichabatsira kuderedza kuitika kwekuwedzera kwepleen-splenomegaly-uye kudiwa kwephlebotomy, nzira yekubvisa ropa rakawanda kubva mumuviri.
Matambudziko anowanzobatanidzwa nekushandiswa kweJakafi mubatori vechikamu ne polycythemia vera mukukwanisa kuongororwa kwekliniki kwaiva nehope yeropa yakawanda (anemia) uye pasi peplate yakawanda (thrombocytopenia). Yakawanda inenge isingave yeropa-nemigumisiro yechirwere yakanga iine chirwere, kubatwa, uye shingles.