Jakafi amaimira chithunzithunzi cha kupita patsogolo mu pharmacology.
Tikukhala m'nthawi zozizwitsa zomwe sitikumvetsetsa bwino njira za matenda komanso momwe tingagwiritsire ntchito njirazi ndi mankhwala atsopano. Mwachitsanzo, Jakafi (ruxolitinib) anakhala mankhwala ovomerezeka a FDA kuti apange polycythemia vera , ndipo amagwira ntchito poletsa maantimicro Janus Associated Kinase 1 (JAK-1) ndi Janus Associated Kinase 2 (JAK-2).
Pogwiritsa ntchito kusintha kwa maselo ena, mavitaminiwa amapita haywire mwa anthu omwe ali ndi polycythemia vera.
Kodi Polycythemia Vera N'chiyani?
Polycythemia vera ndi matenda osadziwika kwambiri a magazi. Ndi matenda osokoneza omwe nthawi zambiri amawonekera m'moyo (anthu a zaka za m'ma 60) ndipo pamapeto pake amachititsa thrombosis (kuganiza molakwika) mwa anthu atatu mwa anthu atatu omwe akukhudzidwa. Monga tonse tikudziwira, kupweteka kumatha kukhala koopsa kotero kuti matenda a PV ndi ovuta kwambiri.
Nkhani ya momwe PV imayambira ntchito m'nthaka. Mafupa athu amachititsa kupanga maselo athu. Mitundu yosiyanasiyana ya maselo m'magazi athu ali ndi maudindo osiyanasiyana. Maselo ofiira amagawira mpweya m'magulu ndi ziwalo zathu, maselo oyera a magazi amathandiza kulimbana ndi matenda ndi mapiritsi omwe amasiya magazi. Kwa anthu omwe ali ndi PV, pali kusintha kwa maselo ambirimbiri omwe amachititsa kuti maselo ofiira a magazi, maselo oyera, ndi mapulateletti azikhala owonjezera. Mwa kuyankhula kwina, mu PV, maselo obadwa, omwe amasiyanitsa m'maselo ofiira ofiira, maselo oyera a magazi, ndi mapiritsi, amaponyedwa mu overdrive.
Chochuluka cha chirichonse sichili chabwino, ndipo pa nkhani ya PV, maselo ochuluka amagazi amatha kuyendetsa mitsempha yathu yomwe imayambitsa mavuto osiyanasiyana a mankhwala monga awa:
- mutu
- kufooka
- pruritis (kuyabwa komwe kumapereka pambuyo pamadzi otentha kapena kusamba)
- chizungulire
- thukuta
- Kuwombera magazi kumatha kutseka mitsempha ndi kuyambitsa kupweteka, kupweteka kwa mtima, ndi kupuma kwa pulmonary, kapena kuvala mitsempha ngati mitsempha yotsegula chiwindi chomwe chimayambitsa chiwindi.)
- magazi (Maselo ambirimbiri a magazi omwe ambiri amakhala opanda mapuloteni-angayambitse magazi)
- splenomegaly (The spleen, yomwe imasungira maselo ofiira a magazi, kutupa chifukwa cha kuchuluka kwa maselo a magazi mu PV.)
- erythromelalgia (Kupweteka komanso kutentha kwa ma chiwerengero zimayambitsidwa ndi mapulogalamu ambiri omwe amachotsa magazi m'mapala ndi zala zomwe zingayambitse chiwerengero cha imfa ndi kudulidwa.)
PV ikhoza kuphatikizanso matenda ena monga matenda a mtima ndi mapiritsi a m'magazi onse chifukwa cha kuchuluka kwa maselo a magazi omwe amayendayenda ndikuyenda bwino kwa minofu hyperplasia kapena kupitirira kumene kumayambitsa magazi. (Minofu yosasuntha imapanga makoma a mitsempha yathu, ndipo kuchuluka kwa maselo a mwazi mwina kumasula zinthu zina zomwe zimachititsa kuti minofu ikhale yofewa.)
Anthu ochepa omwe ali ndi PV amapitirizabe kukhala ndi myelofibrosis (kumene mafupa amathera kapena "atayika" ndipo amadzazidwa ndi fibroblasts osagwira ntchito komanso odzaza magazi omwe amachititsa kuti magazi asapitirire kuchepa kwa magazi). Kumbukirani kuti PV nthawi zambiri imatchedwa myeloproliferative neoplasm kapena khansara chifukwa monga ena khansa amachititsa kuwonjezeka kwa chiwerengero cha nambala ya maselo. Mwamwayi, anthu ena okhala ndi PV, khansa ya m'magazi imayimira mapeto a mzere pa kupitiriza kwa khansara.
Jakafi: Mankhwala Amene Amatsutsana ndi Polycythemia Vera
Anthu omwe ali pulotenti ya PV kapena gawo lomwe amadziwika ndi kuchuluka kwa maselo a magazi amachiritsidwa ndi njira zochepetsera zomwe zimachepetsera zizindikiro komanso kusintha moyo wawo. Ambiri amadziwika bwino pakati pa mankhwalawa ndi phlebotomy kapena magazi kuti athe kuchepetsa chiwerengero cha magazi.
Akatswiri amapatsanso PV ndi myelosuppressive (kuganiza kuti chemotherapeutic) wothandizira-hydroxyurea, busulfan, 32p komanso, posachedwapa, interferon-yomwe imaletsa kuchuluka kwa maselo a magazi. Mankhwala ochiritsa matenda a myelosuppressive amachulukitsa maganizo a wodwalayo ndipo amalingalira kuthandiza anthu okhala ndi PV kukhala moyo wautali.
Mwatsoka, ena mwa mankhwalawa monga chlorambucil ali ndi chiwopsezo choyambitsa khansa ya m'magazi.
Kwa anthu omwe ali ndi PV omwe amalephera kuvomereza kapena osamvera hydroxyurea, jakafi woyendetsa mzere woyamba, Jakafi adavomerezedwa ndi FDA mu December 2014. Jakafi amagwira ntchito poletsa Jak-1 ndi JAK-2 enzyme yomwe imasinthidwa mwa anthu ambiri ndi PV. Mavitaminiwa amagawidwa m'magazi ndi kumagwiritsidwe ntchito kaumunthu, zomwe zimakhala zachilendo kwa anthu omwe ali ndi PV.
Pa 21 peresenti ya anthu omwe sakhala osasamala kapena osamvera hydroxyurea, kafukufuku amasonyeza kuti Jakafi amachepetsa kukula kwa nthonje (amachepetsa splenomegaly) ndipo amachepetsa kufunikira kwa phlebotomy. Kafukufuku akusonyeza kuti ngakhale ndi njira zabwino zothandizira, pali 1 peresenti yokha ya anthu oterowo omwe akanapindulapo. Chodziwika, Jakafi adavomerezedwa kale ndi FDA kuti azitsatira myelofibrosis mu 2011. Zotsatira zovuta kwambiri za Jakafi (zomwe FDA zimatulutsa "zotsatira zoyipa") zikuphatikizapo kuchepa kwa magazi, chiwerengero cha magazi, chizungulire, kudzimbidwa, ndi kumangiriza.
Tiyenera kukumbukira kuti monga momwe ziliri ndi mankhwala ena opatsirana, sikudziwikiratu ngati Jakafi athandiza anthu kukhala moyo wautali.
Ngati inu kapena munthu amene mumamukonda ali ndi PV yomwe sichiyankha kwa hydroxyurea, Jakafi amaimira mankhwala atsopano olonjeza. Kwa ife tonse, Jakafi amaimira chithunzithunzi cha momwe mankhwala ena angapangidwire patsogolo. Ochita kafukufuku akukonzekera bwino pozindikira ndondomeko zomwe zimayambitsidwa ndi matenda ndi kulondolera matendawa.
Zotsatira:
"Thandizo la mayelopliferative neoplasms: liti, ndi liti, ndi motani?" ndi HL Geyer ndi RA Mesa wofalitsidwa mu Magazi pa 12/4/2014.
Prchal JT, Prchal JF. Mutu 86. Polycythemia Vera. Mu: Lichtman MA, Kipps TJ, Seligsohn U, Kaushansky K, Prchal JT. eds. Williams Hematology, 8e . New York, NY: Hill ya McGraw; 2010.